OT Otolaryngology – Head & Neck Surgery Peter Dixon and Ryan Figueroa, chapter editors Jieun Kim and Daniel Soong, associate editors Jef Martin, EBM editor Dr. Jonathan C. Irish and Dr. Evan J. Propst, staf editors

Acronyms ...... 2 Facial Nerve (CN VII) Paralysis ...... 21 Basic Anatomy Review ...... 2 Rhinitis ...... 22 Ear Allergic Rhinitis (Hay Fever) Nose Vasomotor Rhinitis Throat Head and Neck Rhinosinusitis ...... 24 Anatomical Triangles of the Neck Acute Bacterial Rhinosinusitis Chronic Rhinosinusitis Differential Diagnoses of Common Presenting Problems ...... 5 Epistaxis ...... 26 Dizziness Otalgia Hoarseness ...... 27 Hearing Loss Acute Laryngitis Tinnitus Chronic Laryngitis Nasal Obstruction Vocal Cord Polyps Hoarseness Vocal Cord Nodules Neck Mass Benign Laryngeal Papillomas Laryngeal Carcinoma Hearing ...... 9 Normal Hearing Physiology Salivary Glands ...... 29 Types of Hearing Loss Sialadenitis Pure Tone Audiometry Sialolithiasis Speech Audiometry Salivary Gland Neoplasms Impedance Audiometry Parotid Gland Neoplasms Auditory Brainstem Response Otoacoustic Emissions Neck Masses ...... 31 Aural Rehabilitation Approach to a Neck Mass Evaluation Vertigo ...... 12 Evaluation of the Dizzy Patient Congenital Neck Masses ...... 32 Benign Paroxysmal Positional Vertigo Branchial Cleft Cysts/Fistula Menière’s Disease (Endolymphatic Hydrops) Thyroglossal Duct Cysts Vestibular Neuronitis Lymphatic Malformation Labyrinthitis Neoplasms of the Head and Neck ...... 34 Acoustic Neuroma (Vestibular Schwannoma) Thyroid Carcinoma Tinnitus ...... 15 Pediatric Otolaryngology ...... 38 Diseases of the External Ear ...... 15 Acute Otitis Media (AOM) Cerumen Impaction Otitis Media with Effusion (OME) Exostoses Adenoid Hypertrophy Otitis Externa (OE) Adenoidectomy Malignant (Necrotizing) Otitis Externa Sleep-Disordered Breathing in Children (Skull Base Osteomyelitis) Acute Tonsillitis Peritonsillar Abscess (Quinsy) Diseases of the Middle Ear ...... 17 Tonsillectomy Acute Otitis Media and Otitis Media with Airway Problems in Children Effusion Signs of Airway Obstruction Cholesteatoma Acute Laryngotracheobronchitis (Croup) Mastoiditis Acute Epiglottitis Otosclerosis Subglottic Stenosis Laryngomalacia Diseases of the Inner Ear ...... 18 Foreign Body Congenital Sensorineural Hearing Loss Deep Neck Space Infection Presbycusis Sudden Sensorineural Hearing Loss Common Medications ...... 47 Autoimmune Inner Ear Disease Drug Ototoxicity References ...... 48 Noise-Induced Sensorineural Hearing Loss Temporal Bone Fractures

OT1 Otolaryngology Toronto Notes 2014 OT2 Otolaryngology Acronyms/Basic Anatomy Review Toronto Notes 2014

Acronyms

ABR auditory brainstem response FAP familial adenomatous polyposis OSA obstructive sleep apnea AC air conduction FESS functional endoscopic sinus surgery RA rheumatoid arthritis AOM acute otitis media FNA fine needle aspiration SCC squamous cell carcinoma BAHA bone anchored hearing aid GERD gastroesophageal reflux disease SCM sternocleidomastoid BC bone conduction HL hearing loss SNHL sensorineural hearing loss CHL conductive hearing loss HPV human papilloma virus TEF tracheoesophageal fistula CPA cerebellopontine angle INCS intranasal corticosteroids TM tympanic membrane EAC external auditory canal OE otitis externa TNM tumour, node, metastases EBV Epstein-Barr virus OME otitis media with effusion URTI upper respiratory tract infection

Basic Anatomy Review

Ear

External Middle Inner

Temporalis fascia Auditory ossicles Semicircular canals and muscle Helix Malleus Incus Stapes Triangular fossa Vestibular Helical crus nerve Antihelix Vestibulocochlear nerve (CN VIII) Cochlear nerve Scapha Tragus Facial nerve (CN VII)

Cochlea Antiragus

Lobule External acoustic Tympanic Eustachian tube © Aarti Inamdar meatus membrane © Susan Park 2009 Figure 1. Surface anatomy of the external ear; anatomy of ear

Tympanic membrane viewed View into tympanic cavity after through speculum removal of tympanic membrane

Pars flaccida Neck of malleus Lateral process of malleus Incus long process Tensor tympani Stapes tendon Tensor tympani Tendon of muscle stapedius muscle Long process of malleus Umbo (Flat portion) Tympanic plexus Fossa of round (branch of CN IX) (cochlear) window Hypotympanum Cone of light Pars Annulus tensa © Diana Dai 2006 Figure 2. Normal appearance of right tympanic membrane on otoscopy OT3 Otolaryngology Basic Anatomy Review Toronto Notes 2014

Nose

Adenoid

Sphenoid sinus

Superior turbinate Drainage into Nasal Cavity Middle turbinate • Superior meatus: sphenoid (via sphenoethmoidal recess), posterior Middle meatus ethmoid sinuses • Middle meatus: frontal, maxillary, Inferior turbinate anterior ethmoid sinuses Inferior meatus • Inferior meatus: nasolacrimal duct Speculum View of Right Nostril Palatine process of maxilla ine 2003 Ra Soft palate

Opening for Eustachian tube © Jason Figure 3. Nasal anatomy

Anterior ethmoid a. Frontal sinus Posterior ethmoid a.

Sphenoid sinus

Kiesselbach’s plexus Septal branch of sphenopalatine a. Branch of superior labial a. Internal carotid a. Greater palatine a. External carotid a.

Common carotid a. © Barbara Brehovsky 2012

Figure 4. Nasal septum and its arterial supply (see Epistaxis section for detailed blood supply)

Frontal sinus Orbit Ethmoid • Nasopharynx: skull base to soft sinus palate Lamina • Oropharynx: soft palate to hyoid bone papyracea • Laryngopharynx: hyoid bone to Osteomeatal inferior cricoid cartilage complex Maxillary sinus Nasal cavity Teeth

Figure 5. Anatomy of the four paranasal sinuses: maxillary, ethmoid, sphenoid, and frontal Reprinted from Dhillon R.S, and East CA. Ear, Nose and Throat and Head and Neck Surgery, 2nd ed. Copyright 1999, with permission from Elsevier.

Throat

Coronal Section Superior View Valeculla Epiglottis Anterior

Vestibule

Thyroid cartilage

Vestibular folds (false cords) Vocal folds (true cords) Trachea Posterior Pyriform fossa Arytenoid cartilage Posterior View © Glen Oomen 2002 Figure 6. Anatomy of a normal larynx; superior view of larynx on indirect laryngoscopy OT4 Otolaryngology Basic Anatomy Review Toronto Notes 2014

Head and Neck

Temporal branch Zygomatic branch

Buccal branch Superficial temporal a.

Styloid process Maxillary a. Mastoid process Angular a. Stylomastoid foramen Occipital a. Lateral nasal a. Facial n. (VII) Posterior auricular a. Posterior belly of Ascending pharyngeal a. digastric m. Parotid gland Superior labial a. Mandibular branch Internal carotid a. Marginal mandibular Inferior labial a. branch External carotid a.

Cervical branch Facial a. ng 2007

Common carotid a. Wa Lingual a.

Superior thyroid a. © Sean © M. Romanova 2010 © M. Romanova Figure 7. Extratemporal segment of facial nerve Figure 8. Blood supply to the face Branches of facial nerve (in order from superior to inferior) Branches of the external carotid artery (in order from inferior to superior) Ten Zebras Broke My Car Some Angry Lady Figured Out PMS

Hyoid bone

External Thyrohyoid membrane carotid a.

Thyroid cartilage Post. belly Common digastric m. carotid a. bifurcation Common carotid Median cricothyroid ligament Ant. belly a. bifurcation Internal digastric m. jugular v. Sternocleidomastoid m. Hyoid bone Ant. belly Sternocleidomastoid m. omohyoid m. Sternohyoid m. Posterior triangle Omohyoid m. Trapezius m.

Anterior triangle Cricoid cartilage Post. belly omohyoid m. Thyroid gland Sternohyoid m. Trachea Clavicle © Inessa Stanishevskaya 2012 after Figure 9. Anatomy of the neck Anatomical Triangles of the Neck

Anterior triangle: Paired Parasympathetic Ganglia of the Head and Neck • bounded by anterior border of SCM, midline of neck, and lower border of mandible • Ciliary: pupillary constriction • divided into: • Pterygopalatine: lacrimal gland, nasal ƒ submental triangle: bounded by both anterior bellies of digastric and hyoid bone mucosa ƒ digastric triangle: bounded by anterior and posterior bellies of digastric, and inferior border • Submandibular: submandibular, sublingual glands of mandible • Otic: parotid gland ƒ carotid triangle: bounded by sternocleidomastoid, anterior belly of omohyoid, and posterior belly of digastric Š contains: tail of parotid, submandibular gland, hypoglossal nerve, carotid bifurcation, and lymph nodes Function of Facial Nerve Posterior triangle: “Ears, Tears, Face, Taste” • bounded by posterior border of sternocleidomastoid, anterior border of trapezius, and middle Ears: stapedius muscle third of clavicle Tears: lacrimation (lacrimal gland) and • divided into: salivation (parotid) ƒ occipital triangle: superior to posterior belly of the omohyoid Face: muscles of facial expression ƒ Taste: sensory anterior 2/3 of tongue subclavian triangle: inferior to posterior belly of omohyoid (via chorda tympani) • contains: spinal accessory nerve and lymph nodes OT5 Otolaryngology Basic Anatomy Review/Diferential Diagnoses of Presenting Problems Toronto Notes 2014

Table 1. Lymphatic Drainage of Nodal Groups and Anatomical Triangles of Neck Nodal Group/Level Location Drainage 1. Suboccipital (S) Base of skull, posterior Posterior scalp • Left-sided enlargement of a 2. Retroauricular (R) Superficial to mastoid process Scalp, temporal region, external auditory meatus, supraclavicular node (Virchow’s posterior pinna node) may indicate an abdominal 3. Parotid-preauricular (P) In front of ear External auditory meatus, anterior pinna, soft malignancy • Right-sided enlargement may tissue of frontal and temporal regions, root of indicate malignancy of the nose, eyelids, mediastinum, lungs, or esophagus palpebral conjunctiva • Occipital and/or posterior auricular 4. Submental (Level IA) (Midline) Anterior bellies of digastric Floor of mouth, anterior oral tongue, anterior node enlargement may indicate rubella muscles, tip of mandible, and hyoid bone mandibular alveolar ridge, lower lip 5. Submandibular (Level IB) Anterior belly of digastric muscle, Oral cavity, anterior nasal cavity, soft tissues of stylohyoid muscle, body of mandible the mid-face, submandibular gland 6. Upper jugular (Levels IIA Skull base to inferior border of hyoid bone Oral cavity, nasal cavity, naso/oro/hypopharynx, and IIB) along SCM muscle larynx, parotid glands 4 Strap Muscles of the Neck • Thyrohyoid 7. Middle jugular (Level III) Inferior border of hyoid bone to inferior Oral cavity, naso/oro/hypopharynx, larynx • Omohyoid border of cricoid cartilage along SCM • Sternothyroid muscle • Sternohyoid 8. Lower jugular* (Level IV) Inferior border of cricoid cartilage to Hypopharynx, thyroid, cervical esophagus, larynx clavicle along SCM muscle 9. Posterior triangle** Posterior border of SCM, anterior border Nasopharynx and oropharynx, cutaneous STA (Levels VA and VB) of trapezius, from skull base to clavicle structures of the posterior scalp and neck TC CCA 10. Anterior compartment*** (Midline) Hyoid bone to suprasternal Thyroid gland, glottic and subglottic larynx, apex (Level VI) notch of piriform sinus, cervical esophagus IJV CC between the common carotid arteries SPG

*Virchow node: left lower level IV supraclavicular node ITA TG © Marisa Bonofiglio 2003 **Includes some supraclavicular nodes IPG ***Includes pretracheal, precricoid, paratracheal, and perithyroidal nodes RRLN VN (CN X) Differential Diagnoses of Common LRLN Presenting Problems

STA – Superior thyroid artery CCA – Common carotid artery Dizziness IJV – Internal jugular vein ITA – Inferior thyroid artery RRLN – Right recurrent laryngeal nerve Dizziness TC – Thyroid cartilage CC – Cricoid cartilage SPG – Superior parathyroid gland TG – Thyroid gland True Vertigo Non-Vertiginous IPG – Inferior parathyroid gland VN (CN X) – Vagus nerve (CN X) LRLN – Left recurrent laryngeal nerve Peripheral (Vestibular) Central Organic Diseases Functional Figure 10. Anatomy of the thyroid gland

Benign paroxysmal positional Cerebrovascular disorders Cardiac Depression vertigo (BPPV) Vertebrobasiliar insufficiency Arrhythmias Anxiety Labyrinthitis Transient ischemic attacks Aortic stenosis Panic disorder Vestibular neuronitis Wallenberg’s syndrome Vasovagal (hyperventilation) True nystagmus and vertigo caused Menière’s disease Cerebellar infarction Orthostatic hypotension Personality disorder by a peripheral lesion will never last Recurrent vestibulopathy Migrainous vertigo Anemia Phobic dizziness longer than a couple of weeks because Temporal bone fracture Multiple sclerosis Peripheral neuropathy of compensation. Central lesions do Superior semicircular canal dehiscence Tumours Visual impairment not compensate, hence nystagmus and Ototoxic drug exposure CPA tumours vertigo will persist. Autoimmune inner ear disease Posterior fossa tumours Perilymph fistula Glomus tumours Cholesteatoma Inflammation Meningitis Cerebellar abscess Trauma: cerebellar contusion 5 Ds of Vertebrobasilar Insufficiency Common causes in bold Toxic: alcohol, hypnotics, drugs Drop attacks Figure 11. Differential diagnosis of dizziness Diplopia Dysarthria Dizziness Dysphagia OT6 Otolaryngology Diferential Diagnoses of Presenting Problems Toronto Notes 2014

Otalgia

Otalgia

External Ear Middle/Inner Ear Referred Pain

Infection Infection Infection Otitis externa AOM Tonsillitis Herpes simplex/zoster Otitis media with effusion Tracheitis Auricular cellulitis Mastoiditis Ramsay Hunt syndrome External canal abscess Myringitis Trauma Trauma Skull base infections Thyroiditis Frostbite Trauma Cervical arthritis Burns Traumatic perforation Other Hematoma Barotrauma TMJ syndrome Lacerations Other Teeth Other Neoplasm Trismus Neoplasm of external canal Wegener’s Glossopharyngeal neuralgia Foreign body Cholesteatoma Neoplasm of oral cavity, Cerumen impaction larynx, pharynx

Figure 12. Differential diagnosis of otalgia

Hearing Loss

Hearing Loss

Conductive Sensorineural

External Ear Middle Ear Congenital Acquired

Impacted cerumen AOM Genetic Presbycusis Otitis externa Otitis media with effusion Non-syndrome associated Noise-induced Foreign body TM perforation Syndrome associated Menière’s disease Keratosis obturans Otosclerosis Intrauterine infections Labyrinthitis Exostoses, osteomas Tympanosclerosis (i.e. TORCH) Sudden SNHL Tumour of canal Eustachian tube dysfunction Teratogens Autoimmune inner ear disease Congenital stenosis/microtia Cholesteatoma Perinatal hypoxia Ototoxic drug exposure Ossicular malformations Prematurity/low birth weight Temporal bone trauma Ossicular discontinuity Hyperbilirubinemia Infectious Hemotympanum Postmeningitis Middle ear tumour Syphilis Viral: mumps, CMV, HSV Neoplastic Acoustic neuroma CPA tumours Vascular occlusion/emboli Common causes in bold Auditory neuropathy

Figure 13. Differential diagnosis of hearing loss OT7 Otolaryngology Diferential Diagnoses of Presenting Problems Toronto Notes 2014

Tinnitus

Tinnitus

Subjective Objective Only heard by patient Can be heard by others Tinnitus is most commonly associated (common) (rare) with SNHL.

Otologic Vascular Presbycusis Benign intracranial hypertension Noise-induced hearing loss Arteriovenous malformation Otitis media with effusion Glomus tympanicum Menière’s disease Glomus jugulare Otosclerosis Arterial bruits: Cerumen High-riding carotid artery Glomus Tympanicum/Jugulare Tumour Foreign body against TM Vascular loop Signs and Symptoms Drugs Persistent stapedial artery • Pulsatile tinnitus ASA Carotid stenosis • Hearing loss NSAIDs Venous hum: • Blue mass behind TM Aminoglycosides High jugular bulb • Brown’s sign (blanching of the TM Antihypertensives Hypertension with pneumatic otoscopy) Heavy metals Hyper/hypothyroidism Metabolic Mechanical Hyper/hypothyroidism Patulous eustachian tube Hyperlipidemia Palatal myoclonus Vitamin A, B, Zinc deficiency Stapedius muscle spasm Neurologic Head trauma Multiple sclerosis CPA tumours Psychiatric Anxiety Depression Common causes in bold

Figure 14. Differential diagnosis of tinnitus

Nasal Obstruction

Table 2. Differential Diagnosis of Nasal Obstruction Acquired Congenital Nasal Cavity Nasal Cavity • Rhinitis • Nasal dermoid cyst • Acute/chronic • Encephalocele • Vasomotor • Glioma • Allergic • Choanal atresia • Rhinosinusitis • Foreign bodies • Enlarged turbinates • Tumour • Benign: polyps, inverting papilloma • Malignant • SCC • Esthesioneuroblastoma (olfactory neuroblastoma) • Adenocarcinoma Nasal Septum Nasal Septum • Septal deviation • Septal deviation • Septal hematoma/abscess • Septal hematoma/abscess • Dislocated septum • Dislocated septum Nasopharynx • Adenoid hypertrophy • Tumour • Benign: juvenile nasopharyngeal angiofibroma (JNA), polyps • Malignant: nasopharyngeal carcinoma Systemic • Granulomatous diseases, diabetes, vasculitis OT8 Otolaryngology Diferential Diagnoses of Presenting Problems Toronto Notes 2014

Hoarseness

Table 3. Differential Diagnosis of Hoarseness Infectious • Acute/chronic laryngitis • Laryngotracheobronchitis (croup)

Inflammatory • GERD Lung malignancy is the most common • Vocal cord polyps/nodules cause of extralaryngeal vocal cord • Lifestyle: smoking, chronic EtOH use paralysis. Trauma • External laryngeal trauma • Endoscopy and endotracheal tube (e.g. intubation granuloma) Neoplasia • Benign tumour • Malignant tumours (i.e. thyroid) • Papillomas (HPV infection) • SCC • Minor salivary gland tumours • Other • Other Cysts • Retention cysts Systemic • Endocrine • Connective tissue disease • Hypothyroidism • RA • Virilization • SLE Neurologic • Central lesions • Iatrogenic injury – thyroid, parathyroid (vocal cord paralysis due to superior • Cerebrovascular accident (CVA) surgery, carotid endarterectomy, ± recurrent laryngeal nerve injury) • Head injury patent ductus arteriosus (PDA) ligation • Multiple sclerosis (MS) • Bilateral • Skull base tumours • Iatrogenic injury: bilateral thyroid • Arnold-Chiari malformation surgery, forceps delivery • Peripheral lesions • Neuromuscular • Unilateral • Myasthenia gravis • Lung malignancy Functional • Psychogenic aphonia (hysterical aphonia) Congenital • Laryngomalacia • Laryngeal web • Laryngeal atresia

Neck Mass

Neck Mass

Inflammatory/Infections Congenital Neoplastic

Midline Lateral Malignant Benign

Reactive lymphadenopathy Thyroglossal duct cyst Branchial cleft cyst Salivary gland neoplasm TB or atypical mycobacteria Thyroid tumour/goitre Cystic hygroma Lipoma Infectious mononucleosis Pyramidal lobe Fibroma Abscesses of thyroid gland Vascular Cat scratch fever Ranula Sarcoidosis Kawasaki’s HIV Lymphoma Head and neck primary Thyroid Infraclavicular primary Sarcoma Leukemia Salivary gland neoplasm Rhabdomyosarcoma Neuroblastoma

Figure 15. Differential diagnosis of a neck mass OT9 Otolaryngology Hearing Toronto Notes 2014

Hearing

Normal Hearing Physiology

• Conductive pathway (external auditory canal to cochlea): air conduction of sound energy down the EAC vibration of the tympanic membrane (area efect) sequential vibration Order of the Neural Pathway (with of the middle ear ossicles: malleus, incus, stapes (lever efect) transmission of amplifed corresponding waves on ABR) vibrations from the stapes footplate in the middle ear to the oval window of the cochlea in the inner ear pressure diferential on cochlear fuid creates movement along the basilar E COLI Eighth cranial nerve (I – II) membrane within the cochlea from base to apex Cochlear nucleus (III) • Neural pathway (nerve to brain): basilar membrane vibration stimulates overlying hair cells Superior Olivary nucleus in the organ of Corti stimulation of bipolar neurons in the spiral ganglion of the cochlear Lateral leminiscus (IV – V) division of CN VIII cochlear nucleus superior olivary nucleus lateral lemniscus Inferior colliculus inferior colliculus Sylvian fssure of temporal lobe Types of Hearing Loss

1. Conductive Hearing Loss (CHL) • the conduction of sound to the cochlea is impaired • can be caused by external and middle ear disease 2. Sensorineural Hearing Loss (SNHL) • due to a defect in the conversion of sound into neural signals or in the transmission of those signals to the cortex • can be caused by disease of the cochlea, acoustic nerve (CN VIII), brainstem, or cortex 3. Mixed Hearing Loss • both a conductive hearing loss and a sensorineural hearing loss are present Auditory Acuity • whispered-voice test: mask one ear and whisper into the other Weber Test Lateralization = ipsilateral • tuning fork tests (see Table 4) (audiogram is of greater utility) conductive hearing loss or contralateral • sensitivity depends on which tuning fork used (256 Hz, 512 Hz, 1024 Hz) sensorineural hearing loss. ƒ Rinne test: When conductive hearing loss is Š 512 Hz tuning fork is struck and held frmly on mastoid process to test BC. Te tuning present, the Weber test is more fork is then placed beside the pinna to test AC sensitive in detecting the CHL than the Š If AC >BC positive Rinne, which is normal Rinne test. ƒ Weber test: Š 512 Hz tuning fork is held on vertex of head and patient states whether it is heard centrally (Weber negative) or is lateralized to one side (Weber right, Weber lef) Š can place vibrating fork on patient’s chin while they clench their teeth, or directly on HL = Intensity x Duration teeth to elicit more reliable response Š will only lateralize if diference in hearing loss between ears is >6 dB

Table 4. The Interpretation of Tuning Fork Tests

Examples Weber Rinne Frequency of Minimum hearing Normal or bilateral sensorineural hearing loss Central AC>BC (+) bilaterally Tuning Fork loss to have (Hz) NEGATIVE Rinne Right-sided conductive hearing loss, normal left ear Lateralizes to right BC>AC (–) right (BC > AC) (dB) Right-sided sensorineural hearing loss, normal left ear Lateralizes to left AC>BC (+) bilaterally 256 15 512 30 Right-sided severe sensorineural hearing loss or dead right Lateralizes to left BC>AC (–) right* 1024 45 ear, normal left ear * a vibrating tuning fork on the mastoid stimulates the cochlea bilaterally, therefore in this case, the left cochlea is stimulated by the Rinne test on the right, i.e. a false negative test. These tests are not valid if the ear canals are obstructed with cerumen (i.e. will create conductive loss)

Pure Tone Audiometry

• a threshold is the lowest intensity level at which a patient can hear the tone 50% of the time Range of Frequencies Audible to • thresholds are obtained for each ear for frequencies 250 to 8000 Hz Human Ear: • air conduction thresholds are obtained with headphones and measure outer, middle, inner ear, • 20 to 20000 Hz • Most sensitive frequencies: 1000 to and auditory nerve function 4000 Hz • bone conduction thresholds are obtained with bone conduction oscillators which bypass the • Range of human speech: 500 to outer and middle ear 2000 Hz

Degree of Hearing Loss • determined on basis of the pure tone average (PTA) at 500, 1000, and 2000 Hz OT10 Otolaryngology Hearing Toronto Notes 2014

FREQUENCY (Hz) Audiogram Legend 250 500 1000 2000 4000 8000 250 500 1000 2000 4000 8000 for a Left Ear -10 -10 x = AC Unmasked 0 0 Hearing loss most often occurs at 10 10 higher frequencies. Noise-induced > = BC Unmasked 20 LEVEL HEARING 20 (occupational) HL is seen at 4000 Hz. HL = AC Masked 30 30 associated with otosclerosis is seen at ] = BC Masked 40 40 50 50 2000 Hz (Carhart’s notch). 60 60 70 70 80 (dB) 80 90 90 100 100 110 110 Air conduction thresholds can only be 120 120 A. Normal Audiogram B. Conductive Hearing equal to or greater than bone conduction Loss (Otitis Media) thresholds. 250 500 1000 2000 4000 8000 250 500 1000 2000 4000 8000 250 500 1000 2000 4000 8000 -10 -10 -10 0 0 0 10 10 10 20 20 20 30 30 30 40 40 40 50 50 50 60 60 60 70 70 70 80 80 80 90 90 90 100 100 100 110 110 110 120 120 120 C. Conductive Hearing D. Sensorineural E. Sensorineural Loss (Otosclerosis) Hearing Loss Hearing Loss (Noise Induced) (Presbycusis) Figure 16. Types of hearing loss and associated audiograms of a left ear

PURE TONE PATTERNS Degree of Hearing Loss 1. Conductive Hearing Loss (CHL) (Figure 16B and 16C) Decibel Loss Degree of Hearing • BC in normal range Loss • AC outside of normal range 0 to 20 dB Normal • gap between AC and BC thresholds >10 dB (an air-bone gap) 21 to 40 dB Mild 2. Sensorineural Hearing Loss (SNHL) (Figure 16D and 16E) 41 to 55 dB Moderate • both air and bone conduction thresholds below normal 56 to 70 dB Moderate – Severe • gap between AC and BC <10 dB (no air-bone gap) 71 to 90 dB Severe ≥91 dB Profound 3. Mixed Hearing Loss • both air and bone conduction thresholds below normal • gap between AC and BC thresholds >10 dB (an air-bone gap)

Speech Audiometry

Speech Reception Threshold (SRT) • lowest hearing level at which patient is able to repeat 50% of two syllable words which have equal emphasis on each syllable (spondee words) • SRT and best pure tone threshold in the 500 to 2000 Hz range (frequency range of human speech) usually agree within 5 dB. If not, suspect a retrocochlear lesion or functional hearing loss • used to assess the reliability of the pure tone audiometry

Speech Discrimination Test • percentage of words the patient correctly repeats from a list of 50 monosyllabic words • tested at a level 35 to 50 dB > SRT, therefore degree of hearing loss is taken into account • patients with normal hearing or conductive hearing loss score >90% • score depends on extent of SNHL • rollover efect: a decrease in discrimination as sound intensity increases. Typical of a retrocochlear lesion (e.g. acoustic neuroma) • investigate further if scores difer more than 20% between ears as asymmetry may indicate a retrocochlear lesion • used as best predictor of hearing aid response: if patient has HL and problems with word discrimination, hearing aids may not be helpful OT11 Otolaryngology Hearing Toronto Notes 2014

Impedance Audiometry

Tympanogram • the Eustachian tube equalizes the pressure between the external and middle ear • tympanograms graph the compliance of the middle ear system against a pressure gradient ranging from to –400 to +200 mmH2O • tympanogram peak occurs at the point of maximum compliance: where the pressure in the external canal is equivalent to the pressure in the middle ear • normal range: –100 to +50 mmH2O

High Type A Type B Type C Compliance

Low – O+ – O+ – O+ Air Pressure Air Pressure Air Pressure • Normal pressure peak at 0 • No pressure peak • Negative pressure peak • Note: with otosclerosis, peak is still at • Poor TM mobility indicative of • Indicative of Eustachian tube dysfunction 0 mmH2O but has a lower amplitude middle ear effusion (OME) or or early stage otitis media without effusion • Note: with ossicular chain discontinuity, perforated TM

peak is still at 0 mmH2O but has a higher amplitude Figure 17. Tympanograms

Static Compliance • volume measurement refecting overall stifness of the middle ear system • normal range: 0.3 to 1.6 cc • negative middle ear pressure and abnormal compliance indicate middle ear pathology • in a type B curve, ear canal volumes of greater than 2 cc in children and 2.5 cc in adults indicate TM perforation or presence of a patent ventilation tube

Acoustic Stapedial Reflexes • stapedius muscle contracts due to loud sound • acoustic refex thresholds = 70 to 100 dB greater than hearing threshold; if hearing threshold >85 dB, refex likely absent • stimulating either ear causes bilateral and symmetrical refexes • for refex to be present, CN VII must be intact and no conductive hearing loss in monitored ear • if refex is absent without conductive or severe sensorineural loss, suspect CN VII lesion • acoustic refex decay test = ability of stapedius muscle to sustain contraction for 10 s at 10 dB • normally, little refex decay occurs at 500 and 1000 Hz • with cochlear hearing loss, acoustic refex thresholds are 25 to 60 dB • with retrocochlear hearing loss (acoustic neuroma), absent acoustic refexes or marked refex decay (>50%) within 5 s

Auditory Brainstem Response (ABR)

• measures neuroelectric potentials (waves) in response to a stimulus in fve diferent anatomic sites (refer to Order of Neural Pathway sidebar on OT9). Tis test can be used to map the lesion according to the site of the defect • delay in brainstem response suggests cochlear or retrocochlear abnormalities • does not require volition or co-operation of patient (therefore of value in children and in malingerers) Otoacoustic Emissions

• objective test of hearing where a series of clicks is presented to the ear and the cochlea generates an echo which can be measured • ofen used in newborn screening • can be used to uncover normal hearing in malingering patients • absence of emissions can be due to hearing loss or fuid in the middle ear OT12 Otolaryngology Hearing/Vertigo Toronto Notes 2014

Aural Rehabilitation

• dependent on degree of hearing loss, communicative requirements, motivation, expectations, Pre-lingual deafness: deafness occurring before speech and language age, and physical and mental abilities are acquired. • negative prognostic factors: Post-lingual deafness: deafness ƒ poor speech discrimination occurring after speech and language ƒ narrow dynamic range (recruitment) are acquired. ƒ unrealistic expectations • types of hearing aids: ƒ BTE: behind the ear (with occlusive mould or open ft which allows natural sound to pass – for milder hearing losses) Pre-lingually deaf infants are the best ƒ ITE: in-the-ear, placed in concha candidates for aural rehabilitation ƒ ITC: in-the-canal, placed entirely in ear canal because they have maximal benefit from ƒ CIC: contained-in-canal, placed deeply in ear canal ongoing developmental plasticity. ƒ bone conduction – bone-anchored hearing aid (BAHA): attached to the skull ƒ contralateral routing of signals (CROS) • assistive listening devices: ƒ direct/indirect audio output Bone Anchored Hearing Aids (BAHA) ƒ infrared, FM radio, or induction loop systems BAHAs function based on bone ƒ telephone, television, or alerting devices conduction and are indicated primarily • cochlear implants: for patients with conductive hearing ƒ electrode is inserted into the cochlea to allow direct stimulation of the auditory nerve loss, unilateral hearing loss, and ƒ for profound bilateral sensorineural hearing loss not rehabilitated with conventional hearing aids mixed hearing loss who cannot wear ƒ conventional hearing aids. BAHAs established indication: post-lingually deafened adults, pre- and post-lingually deaf children consist of a titanium implant, an external abutment, and a sound processor. The sound processor transmits vibrations Vertigo through the external abutment to the titanium implant and then directly to the cochlea. Evaluation of the Dizzy Patient

• vertigo: illusion of rotational, linear, or tilting movement of self or environment ƒ vertigo is produced by peripheral (inner ear) or central (brainstem-cerebellum) stimulation • it is important to distinguish vertigo from other disease entities that may present with similar complaints of “dizziness” (e.g. cardiovascular, psychiatric, neurological, aging)

Table 5. Peripheral vs. Central Vertigo Symptoms Peripheral Central Imbalance Moderate-severe Mild-moderate Nausea and vomiting Severe Variable Auditory symptoms Common Rare Neurologic symptoms Rare Common Compensation Rapid Slow Nystagmus Unidirectional Bidirectional Horizontal or rotatory Horizontal or vertical

Table 6. Differential Diagnosis of Vertigo Based on History Condition Duration Hearing Loss Tinnitus Aural Fullness Other Features Benign paroxysmal Seconds – – – positional vertigo (BPPV) Menière’s disease Minutes to hours Uni/bilateral, + Pressure/warmth Precedes attack fluctuating Vestibular neuronitis Hours to days – – – Labyrinthitis Days Unilateral Whistling – Recent AOM Acoustic neuroma Chronic Progressive + – Ataxia CN VII palsy

Benign Paroxysmal Positional Vertigo (BPPV)

Definition • acute attacks of transient vertigo lasting seconds to minutes initiated by certain head positions, BPPV is the most common cause of accompanied by torsional (i.e. rotatory) nystagmus (geotropic = fast phase towards the foor) episodic vertigo. Patients often are symptomatic when Etiology rolling over in bed or moving their • due to canalithiasis (migration of free foating otoliths within the endolymph of the semicircular head to a position of extreme posterior canal) or cupulolithiasis (otolith attached to the cupula of the semicircular canal) extension such as looking up at a tall ƒ building or getting their hair washed at can afect each of the 3 semicircular canals, although the posterior canal is afected in >90% the hairdresser. of cases OT13 Otolaryngology Vertigo Toronto Notes 2014

ƒ causes: head injury, viral infection (URTI), degenerative disease, idiopathic ƒ results in slightly diferent signals being received by the brain from the two balance organs resulting in sensation of movement Patients can wear Frenzel’s magnifying eyeglasses during the Dix-Hallpike Diagnosis maneuver, which inhibit visual fixation and allow for better visualization of the • history eyes. • positive Dix-Hallpike maneuver (sensitivity 82%, specifcity 71%)

Dix-Hallpike Positional Testing (see website for video and illustrations) • the patient is rapidly moved from a sitting position to a supine position with the head hanging over the end of the table, turned to one side at 45° and neck extended 20° holding the position for 20 s • onset of vertigo and rotary nystagmus indicate a positive test for the dependent side 5 Signs of BPPV Seen with Dix-Hallpike Maneuver Treatment • Geotropic rotatory nystagmus • reassure patient that process resolves spontaneously (nystagmus MUST be present for a • particle repositioning maneuvers positive test) ƒ Epley maneuver (performed by MD) • Fatigues with repeated maneuver and ƒ fixation Brandt-Darof exercises (performed by patient) • Reversal of nystagmus upon sitting up • surgery for refractory cases • Latency of ~20 s • anti-emetics for nausea/vomiting • Crescendo/decrescendo vertigo • drugs to suppress the vestibular system delay eventual recovery and are therefore not used lasting 20 s Menière’s Disease (Endolymphatic Hydrops)

Definition Diagnostic Criteria for Menière’s • episodic attacks of tinnitus, hearing loss, aural fullness, and vertigo lasting minutes to hours Disease (must have all three): • Two spontaneous episodes of Proposed Etiology rotational vertigo ≥20 minutes • Audiometric confirmation of SNHL • inadequate absorption of endolymph leads to endolymphatic hydrops (over accumulation) that (often low frequency) distorts the membranous labyrinth • Tinnitus and/or aural fullness Epidemiology • peak incidence 40 to 60 yr • bilateral in 35% of cases Drop Attacks (Tumarkin’s Otolithic Clinical Features Crisis) are sudden falls occurring without warning and without LOC. • vertigo, fuctuating low frequency SNHL, tinnitus, and aural fullness • ± drop attacks (Tumarkin crisis), ± nausea and vomiting • vertigo disappears with time (minutes to hours), but hearing loss remains • early in the disease: fuctuating SNHL • later stages: persistent tinnitus and progressive hearing loss • attacks come in clusters and can be debilitating to the patient Before proceeding with gentamicin • triggers: high salt intake, cafeine, stress, nicotine, and alcohol treatment, perform a gadolinium enhanced MRI to rule out CPA tumour as Treatment the cause of symptoms. • acute management may consist of bed rest, antiemetics, antivertiginous drugs [e.g. betahistine (Serc®)], and low molecular weight dextrans (not commonly used) • long term management may include: ƒ medical: Š low salt diet, diuretics (e.g. hydrochlorothiazide, triamterene, amiloride) Š Serc® prophylactically to decrease intensity of attacks Š local application of gentamicin to destroy vestibular end-organ, results in complete SNHL ƒ surgical: Š selective vestibular neurectomy or transtympanic labyrinthectomy Š vestibular implants have recently been introduced, experimentally • must monitor opposite ear as bilaterality occurs in 35% of cases Vestibular Neuronitis

Definition • acute onset of disabling vertigo ofen accompanied by nausea, vomiting, and imbalance without hearing loss that resolves over days leaving a residual imbalance that lasts days to weeks Etiology • thought to be due to a viral infection (e.g. measles, mumps, herpes zoster) • ~30% of cases have associated URTI symptoms • other: microvascular events, diabetes, autoimmune process • considered to be the vestibular equivalent of Bell’s palsy, sudden hearing loss, and acute vocal cord palsy

OT14 Otolaryngology Vertigo Toronto Notes 2014

Clinical Features • acute phase: ƒ severe vertigo with nausea, vomiting, and imbalance lasting 1 to 5 d ƒ irritative nystagmus (fast phase towards the ofending ear) ƒ patient tends to veer towards afected side • convalescent phase: ƒ imbalance and motion sickness lasting days to weeks ƒ spontaneous nystagmus away from afected side ƒ gradual vestibular adaptation requires weeks to months • incomplete recovery likely with the following risk factors: elderly, visual impairment, poor ambulation • repeated attacks can occur Treatment • acute phase: ƒ bed rest, vestibular sedatives (Gravol®), diazepam • convalescent phase: ƒ progressive ambulation especially in the elderly ƒ vestibular exercises: involve eye and head movements, sitting, standing, and walking

Labyrinthitis

Definition • acute infection of the inner ear resulting in vertigo and hearing loss Etiology • may be serous (viral) or purulent (bacterial) • occurs as a complication of acute and chronic otitis media, bacterial meningitis, cholesteatoma, and temporal bone fractures • bacterial: S. pneumoniae, H, infuenzae, M. catarrhalis, P. aeruginosa, P. mirabilis • viral: rubella, CMV, measles, mumps, varicella zoster Clinical Features • sudden onset of vertigo, nausea, vomiting, tinnitus, and unilateral hearing loss, with no associated fever or pain • meningitis is a serious complication Investigations • CT head • if meningitis is suspected: lumbar puncture, blood cultures Treatment • treat with IV antibiotics, drainage of middle ear ± mastoidectomy

Acoustic Neuroma (Vestibular Schwannoma)

Definition • schwannoma of the vestibular portion of CN VIII Pathogenesis • starts in the internal auditory canal and expands into cerebellopontine angle (CPA), compressing cerebellum and brainstem • when associated with type 2 neurofbromatosis (NF2): bilateral acoustic neuromas, café-au-lait skin lesions, and multiple intracranial lesions Clinical Features • usually presents with unilateral SNHL or tinnitus Acoustic neuroma is the most common • dizziness and unsteadiness may be present, but true vertigo is rare as tumour growth occurs intracranial tumour causing SNHL slowly and thus compensation occurs and the most common cerebellopontine angle tumour. • facial nerve palsy and trigeminal (V1) sensory defcit (corneal refex) are late complications Diagnosis • MRI with gadolinium contrast is the gold standard • audiogram (to assess SNHL) • poor speech discrimination relative to the hearing loss In the elderly, unilateral tinnitus or • stapedial refex absent or signifcant refex decay SNHL is acoustic neuroma until proven • ABR – increase in latency of the 5th wave otherwise. • vestibular tests: normal or asymmetric caloric weakness (an early sign) Treatment • expectant management if tumour is very small, or in elderly • defnitive management is surgical excision • other options: gamma knife, radiation OT15 Otolaryngology Tinnitus/Diseases of the External Ear Toronto Notes 2014

Tinnitus

Definition • an auditory perception in the absence of an acoustic stimuli, likely related to loss of input to neurons in central auditory pathways and resulting in abnormal fring

History • subjective vs. objective (see Figure 14, OT7) • continuous vs. pulsatile (vascular in origin) • unilateral vs. bilateral • associated symptoms: hearing loss, vertigo, aural fullness, otalgia, otorrhea

Investigations • audiology • if unilateral: ƒ ABR, gadolinium enhanced MRI to exclude a retrocochlear lesion ƒ CT to diagnose glomus tympanicum (rare) ƒ MRI or angiogram to diagnose AVM • if suspect metabolic abnormality: lipid profle, TSH

Treatment • if a cause is found, treat the cause (e.g. drainage of middle ear efusion, embolization or excision of AVM) • with no treatable cause: 50% will improve, 25% worsen, 25% remain the same • avoid loud noise, ototoxic meds, cafeine, smoking • tinnitus clinics • identify situations where tinnitus is most bothersome (e.g. quiet times), mask tinnitus with sof music or “white noise” • hearing aid if coexistent hearing loss • tinnitus instrument: combines hearing aid with white noise masker • trial of tocainamide Diseases of the External Ear

Cerumen Impaction

Etiology Cerumen impaction is the most common cause of conductive hearing loss for • ear wax is a mixture of secretions from ceruminous and pilosebaceous glands, squames of those aged 15-50 yr. epithelium, dust, and debris Risk Factors • hairy or narrow ear canals, in-the-ear hearing aids, cotton swab usage, osteomata Clinical Features • hearing loss (conductive) • ± tinnitus, vertigo, otalgia, aural fullness Syringing Indications: Treatment • Totally occlusive cerumen with pain, • ceruminolytic drops (bicarbonate solution, olive oil, glycerine, Cerumenol®, Cerumenex®) decreased hearing, or tinnitus • syringing Contraindications: • manual debridement (by MD) • Active infection • Previous ear surgery • Only hearing ear Exostoses • TM perforation Complications: • Otitis externa Definition • TM perforation • bony protuberances in the external auditory canal composed of lamellar bone • Trauma • Pain Etiology • Vertigo • Tinnitus • possible association with swimming in cold water • Otitis media Method: Clinical Features • Establish that TM is intact • usually an incidental fnding • Gently pull the pinna superiorly and • if large, they can cause cerumen impaction or otitis externa posteriorly • Using warm water, aim the syringe nozzle upwards and posteriorly to Treatment irrigate the ear canal • no treatment required unless symptomatic OT16 Otolaryngology Diseases of the External Ear Toronto Notes 2014

Otitis Externa (OE)

Etiology • bacteria (~90% of OE): Pseudomonas aeruginosa, Pseudomonas vulgaris, E. coli, S. aureus • fungus: Candida albicans, Aspergillus niger

Risk Factors • associated with swimming (“swimmer’s ear”) • mechanical cleaning (Q-tips®), skin dermatitis, aggressive scratching • devices that occlude the ear canal: hearing aids, headphones, etc.

Clinical Features • acute: ƒ pain aggravated by movement of auricle (traction of pinna or pressure over tragus) ƒ otorrhea (sticky yellow purulent discharge) ƒ conductive hearing loss ± aural fullness 2º to obstruction of external canal by swelling and Pulling on the pinna is extremely painful purulent debris in otitis externa, but is usually well ƒ post-auricular lymphadenopathy tolerated in otitis media. ƒ complicated OE exists if the pinna and/or the periauricular sof tissues are erythematous and swollen • chronic: ƒ pruritus of external ear ± excoriation of ear canal ƒ atrophic and scaly epidermal lining, ± otorrhea, ± hearing loss ƒ wide meatus but no pain with movement of auricle ƒ tympanic membrane appears normal

Treatment • clean ear under magnifcation with irrigation, suction, dry swabbing, and C&S • bacterial etiology ƒ antipseudomonal otic drops (e.g. ciprofoxacin) or a combination of antibiotic and steroid (e.g. Cipro HC®) ƒ do not use aminoglycoside if the tympanic membrane (TM) is perforated because of the risk of ototoxicity ƒ introduction of fne gauze wick (pope wick) if external canal edematous ƒ ± 3% acetic acid solution to acidify ear canal (low pH is bacteriostatic) ƒ systemic antibiotics if either cervical lymphadenopathy or cellulitis is present • fungal etiology ƒ repeated debridement and topical antifungals (gentian violet, Mycostatin® powder, boric acid, Locacorten®, Vioform® drops) • ± analgesics • chronic otitis externa (pruritus without obvious infection) corticosteroid alone (e.g. diprosalic acid) Malignant (Necrotizing) Otitis Externa (Skull Base Osteomyelitis)

Definition • osteomyelitis of the temporal bone

Epidemiology • occurs in elderly diabetics and immunocompromised patients

Etiology • rare complication of otitis externa • Pseudomonas infection in 99% of cases

Clinical Features Gallium and Technetium Scans • otalgia and purulent otorrhea that is refractory to medical therapy Gallium scans are used to show sites • granulation tissue on the foor of the auditory canal of active infection. Gallium is taken up by PMNs and therefore only lights up when active infection is present. It will Complications not show the extent of osteomyelitis. • cranial nerve palsies (most commonly VII>X>XI) Technetium scans provide information • systemic infection, death about osteoblastic activity and as such are used to demonstrate sites of osteomyelitis. Technetium scans help Management with diagnosis whereas gallium scans • imaging: high resolution temporal bone CT scan, gadolinium enhanced MRI, technetium scan are useful in follow-up. • requires hospital admission, debridement, IV antibiotics, hyperbaric O2 • may require OR for debridement of necrotic tissue/bone OT17 Otolaryngology Diseases of the Middle Ear Toronto Notes 2014

Diseases of the Middle Ear

Acute Otitis Media (AOM) and Otitis Media with Effusion (OME) • see Pediatric Otolaryngology, OT38

Cholesteatoma

Definition • a cyst composed of keratinizing squamous epithelium occurring in the middle ear, mastoid, and temporal bone • two types: congenital and acquired

Congenital • presents as a “small white pearl” behind an intact tympanic membrane (anterior and medial to the malleus) or as a conductive hearing loss • believed to be due to aberrant migration of external canal ectoderm during development Mechanisms of Cholesteatoma • not associated with otitis media/Eustachian tube dysfunction Formation • Epithelial migration through TM perforation (2° acquired) Acquired (more common) • Invagination of TM (1° acquired) • generally occurs as a consequence of otitis media and chronic Eustachian tube dysfunction • Metaplasia of middle ear epithelium or • frequently associated with retraction pockets in the pars faccida (1° acquired) and marginal basal cell hyperplasia (congenital) perforations (2° acquired) of the tympanic membrane • the associated chronic infammatory process causes progressive destruction of surrounding bony structures

Clinical Features • symptoms: ƒ history of otitis media (especially if unilateral), ventilation tubes, ear surgery ƒ progressive hearing loss (predominantly conductive although may get sensorineural hearing loss in late stage) ƒ otalgia, aural fullness, fever • signs: ƒ retraction pocket in TM, may contain keratin debris ƒ TM perforation ƒ granulation tissue, polyp visible on otoscopy ƒ malodorous, unilateral otorrhea

Complications

Table 7. Complications of Cholesteatoma Local Intracranial Ossicular erosion: conductive hearing loss Meningitis Inner ear erosion: SNHL, dizziness, and/or labyrinthitis Sigmoid sinus thrombosis Temporal bone infection: mastoiditis, petrositis Intracranial abscess (subdural, epidural, cerebellar) Facial paralysis

Investigations • audiogram and CT scan

Treatment • there is no conservative therapy for cholesteatoma • surgical: mastoidectomy ± tympanoplasty ± ossicular reconstruction

Mastoiditis

Definition • infection (usually subperiosteal) of mastoid air cells, most commonly seen approximately two weeks afer onset of untreated or inadequately treated acute suppurative otitis media Complications of AOM are rare due to rapid and effective treatment of AOM Etiology with antibiotics. • acute mastoiditis caused by the same organisms as AOM: S. pneumoniae, H. infuenzae, M. catarrhalis, S. pyogenes, S. aureus, P. aeruginosa

OT18 Otolaryngology Diseases of the Middle Ear/Diseases of the Inner Ear Toronto Notes 2014

Clinical Features • otorrhea • tenderness to pressure over the mastoid Classic Triad • retroauricular swelling with protruding ear • Otorrhea • fever, hearing loss, ± TM perforation (late) • Tenderness to pressure over the • CT radiologic fndings: opacifcation of mastoid air cells by fuid and interruption of normal mastoid • Retroauricular swelling with trabeculations of cells (coalescence) protruding ear Treatment • IV antibiotics with myringotomy and ventilation tubes – usually all that is required acutely • cortical mastoidectomy: ƒ debridement of infected tissue allowing aeration and drainage • indications for surgery: ƒ failure of medical treatment afer 48 h ƒ symptoms of intracranial complications ƒ aural discharge persisting for 4 wk and resistant to antibiotics

Otosclerosis

Definition • fusion of stapes footplate to oval window so that it cannot vibrate

Etiology • autosomal dominant, variable penetrance approximately 40% • female > male, progresses during pregnancy (hormone responsive)

Clinical Features • progressive conductive hearing loss frst noticed in teens and 20s (may progress to sensorineural hearing loss if cochlea involved) Otosclerosis is the 2nd most common • ± pulsatile tinnitus cause of conductive hearing loss in • tympanic membrane normal ± pink blush (Schwartz’s sign) associated with the 15 to 50 year olds (after cerumen neovascularization of otosclerotic bone impaction). • characteristic dip at 2000 Hz (Carhart’s notch) on audiogram (see Figure 16C, OT10)

Treatment • monitor with serial audiograms if coping with loss • hearing aid (air conduction, bone conduction, BAHA) • stapedectomy or stapedotomy (with laser or drill) with prosthesis is defnitive treatment Diseases of the Inner Ear

Congenital Sensorineural Hearing Loss

Hereditary Defects • non-syndrome associated (70%): ƒ ofen idiopathic, autosomal recessive ƒ connexin 26 (GJB2) most common • syndrome associated (30%): ƒ Waardenburg: white forelock, heterochromia iridis (each eye diferent color), wide nasal bridge and increased distance between medial canthi ƒ Pendred: deafness associated with thyroid gland disorders, SLC26A4 gene, enlarged vestibular aqueducts ƒ Treacher-Collins: frst and second branchial clef anomalies ƒ Alport: hereditary nephritis Prenatal TORCH Infections • toxoplasmosis, rubella, CMV, herpes simplex, others (e.g. HIV, syphilis) Perinatal • Rh incompatibility • anoxia • hyperbilirubinemia • birth trauma (hemorrhage into inner ear) Postnatal • meningitis, mumps, measles OT19 Otolaryngology Diseases of the Inner Ear Toronto Notes 2014

High Risk Factors (for Hearing Loss in Newborns) • low birth weight/prematurity • perinatal anoxia (low APGARs) • kernicterus: bilirubin >25 mg/dL • craniofacial abnormality • family history of deafness in childhood • 1st trimester illness: TORCH infections • neonatal sepsis • ototoxic drugs • perinatal infection, including post-natal meningitis • consanguinity • 50-75% of newborns with sensorineural hearing loss have at least one of the above risk factors, and 90% of these have spent time in the NICU • presence of any risk factor: ABR study performed before leaving NICU and at 3 mo adjusted age • early rehabilitation improves speech and school performance

Presbycusis

Definition • sensorineural hearing loss associated with aging (starting in 5th and 6th decades)

Etiology • hair cell degeneration • age related degeneration of basilar membrane, possibly genetic etiology • cochlear neuron damage • ischemia of inner ear

Clinical Features • progressive, bilateral hearing loss initially at high frequencies, then middle frequencies • loss of discrimination of speech especially with background noise present – patients describe people as mumbling Presbycusis is the most common cause • recruitment phenomenon: inability to tolerate loud sounds of SNHL. • tinnitus

Treatment • hearing aid if patient has difculty functioning, hearing loss >30-35 dB, and good speech discrimination • ± lip reading, auditory training, auditory aids (doorbell and phone lights)

Sudden Sensorineural Hearing Loss

Clinical Features • presents as a sudden onset of signifcant SNHL (usually unilateral) ± tinnitus, aural fullness • usually idiopathic, rule out other causes: Sudden sensorineural hearing loss ƒ autoimmune causes (e.g. ESR, rheumatoid factor, ANA) may easily be confused with ischemic ƒ MRI to rule out tumour and/or CT to rule out ischemic/hemorrhagic stroke if associated brain events. It is important to keep a high index of suspicion especially with with any other focal neurological signs (e.g. vertigo, ataxia, abnormality of CN V or VII, elderly patients presenting with sudden weakness) sensorineural hearing loss as well as vertigo. Treatment • oral corticosteroids within 3 d of onset: prednisone 1 mg/kg/d for 10-14 d

Prognosis • depends on degree of hearing loss • 70% resolve within 10 to 14 d • 20% experience partial resolution • 10% experience permanent hearing loss

Autoimmune Inner Ear Disease

Etiology • idiopathic • may be associated with systemic autoimmune diseases (i.e. rheumatoid arthritis, SLE), vasculitides (i.e. granulomatosis with polyangiitis, polyarteritis nodosa) and allergies

Epidemiology • most common between ages 20-50 OT20 Otolaryngology Diseases of the Inner Ear Toronto Notes 2014

Clinical Features • rapidly progressive or fuctuating bilateral SNHL • ± tinnitus, aural fullness, vestibular symptoms (i.e. ataxia, disequilibrium, vertigo)

Investigations • autoimmune work-up: CBC, ESR, ANA, rheumatoid factor

Treatment • high-dose corticosteroids: treat early for at least 30 d • consider cytotoxic medication for steroid non-responders

Drug Ototoxicity

Aminoglycosides • streptomycin and gentamicin (vestibulotoxic), kanamycin and tobramycin (cochleotoxic) • toxic to hair cells by any route: oral, IV, and topical (if the TM is perforated) • destroys sensory hair cells: outer frst, inner second (therefore otoacoustic emissions are lost frst) • high frequency hearing loss develops earliest • ototoxicity occurs days to weeks post-treatment • must monitor with peak and trough levels when prescribed, especially if patient has neutropenia and/or history of ear or renal problems • q24h dosing recommended (with amount determined by creatinine clearance) • aminoglycoside toxicity displays saturable kinetics therefore once daily dosing presents less risk than divided daily doses • duration of treatment is the most important predictor of ototoxicity • treatment: immediately stop aminoglycosides

Salicylates • hearing loss with tinnitus, reversible if discontinued

Antimalarials (Quinines) • hearing loss with tinnitus • reversible if discontinued but can lead to permanent loss

Others • many antineoplastic agents are ototoxic (weigh risks vs. benefts) • loop diuretics

Noise-Induced Sensorineural Hearing Loss

Pathogenesis • 85 to 90 dB over months or years or single sound impulses >135 dB can cause cochlear damage • bilateral SNHL initially and most prominently at 4000 Hz (resonant frequency of the temporal bone), known as “boilermaker’s notch” on audiogram, extends to higher and lower frequencies with time (see Figure 16D, OT10) • speech reception not altered until hearing loss >30 dB at speech frequency, therefore considerable damage may occur before patient complains of hearing loss • difculty with speech discrimination, especially in situations with competing noise

Phases of Hearing Loss • dependent on: intensity of sound and duration of exposure • temporary threshold shif: ƒ when exposed to loud sound, decreased sensitivity or increased threshold for sound ƒ may have associated aural fullness and tinnitus ƒ with removal of noise, hearing returns to normal • permanent threshold shif: ƒ hearing does not return to previous state

Treatment • hearing aid • prevention: ƒ ear protectors: mufs, plugs ƒ limit exposure to noise with frequent rest periods ƒ regular audiologic follow-up OT21 Otolaryngology Diseases of the Inner Ear/Facial Nerve (CN VII) Paralysis Toronto Notes 2014

Temporal Bone Fractures

Table 8. Features of Temporal Bone Fractures (see Figure 18) Transverse (1) Longitudinal (2) Extension Into bony labyrinth and internal auditory meatus Into middle ear Incidence 10 to 20% 70 to 90% Etiology Frontal/occipital trauma Lateral skull trauma CN pathology CN VII palsy (50%) CN VII palsy (10 to 20%) 1 Hearing loss Sensorineural loss due to direct cochlear injury Conductive hearing loss secondary to ossicular 2 injury Vestibular symptoms Sudden onset vestibular symptoms due to Rare direct semicircular canal injury (vertigo, spontaneous nystagmus) Other features Intact external auditory meatus, tympanic Torn tympanic membrane or hemotympanum membrane ± hemotympanum Bleeding from external auditory canal © Teddy Cameron 2002 Spontaneous nystagmus Step formation in external auditory canal Figure 18. Types of temporal bone CSF leak in Eustachian tube to nasopharynx CSF otorrhea fractures ± rhinorrhea (risk of meningitis) Battle’s sign = mastoid ecchymoses Raccoon eyes = periorbital ecchymoses

• characterized as longitudinal or transverse relative to the long axis of the petrous temporal bone • rarely are temporal bone fractures purely transverse or longitudinal, ofen it is a mixed picture Signs of Basilar Skull Fracture Battle’s Sign: ecchymosis of the Diagnosis mastoid process of the temporal bone • otoscopy Racoon Eyes • do not syringe or manipulate external auditory meatus due to risk of inducing meningitis via CSF Rhinorrhea/Otorrhea TM perforation Cranial Nerve Involvement: facial palsy • CT head CN VII, nystagmus CN VI, facial • audiology, facial nerve tests (for transverse fractures), Schirmer’s test (of lacrimation), stapedial numbness CN V refexes if CN VII palsy • if suspecting CSF leak: look for halo sign, send fuid for -2 transferrin

Treatment The halo sign: the double ringed • ABCs appearance of CSF fluid on white filter • medical – expectant, prevent otogenic meningitis paper as it separates out from blood. • surgical – explore temporal bone, indications: ƒ CN VII palsy (immediate and complete) ƒ gunshot wound ƒ depressed fracture of external auditory meatus Hemotympanum can be indicative of ƒ early meningitis (mastoidectomy) temporal bone trauma. ƒ bleeding intracranially from sinus ƒ CSF otorrhea (may resolve spontaneously)

Complications • acute otitis media ± labyrinthitis ± mastoiditis • meningitis/epidural abscess/brain abscess • post-traumatic cholesteatoma

House-Brackmann Facial Nerve Facial Nerve (CN VII) Paralysis Grading System Grade I: Normal facial motor function Grade II: Mild dysfunction Etiology • Slight weakness • supranuclear and nuclear (MS, poliomyelitis, cerebral tumours) • Normal symmetry and tone at rest • infranuclear (see Table 9) • Complete eye closure Grade III: Moderate dysfunction Treatment • Obvious weakness • treat according to etiology plus provide corneal protection with artifcial tears, nocturnal lid Grade IV: Moderately severe dysfunction • Obvious weakness taping, tarsorrhaphy, gold weighting of upper lid ± disfiguring asymmetry • facial paralysis that does not resolve with time or with medical treatment will ofen be referred • Incomplete eye closure for possible reanimation techniques to restore function • No forehead motion ƒ common reanimation techniques include: • Mouth asymmetric motion Š Grade V: Severe dysfunction direct facial nerve anastomosis • Barely perceptible motion of Š interpositional grafs mouth Š anastomosis to other motor nerves • Asymmetric at rest Š muscle transpositions Grade VI: Total paralysis • No movement OT22 Otolaryngology Facial Nerve (CN VII) Paralysis/Rhinitis Toronto Notes 2014

Table 9. Differential Diagnosis of Peripheral Facial Paralysis (PFP)

Etiology Incidence Findings Investigations Treatment, Follow-up, and Prognosis (Px) Bell’s Palsy 80 to 90% of PFP Hx: Stapedial reflex absent Rx: Idiopathic, Risk Factors: Acute onset Audiology normal (or Protect the eye to prevent exposure keratitis with (HSV) infection Diabetes Numbness of ear baseline) patching or tarsorraphy of the facial nerve Pregnancy Schirmer’s test EMG – best measure for Systemic steroids may lessen degeneration and hasten Diagnosis of exclusion Viral prodrome Recurrence (12%) prognosis recovery (50%) + FHx (14%) Topognostic testing Consider antiviral (acyclovir) Hyperacusis (30%) MRI with gadolinium – F/U: P/E: enhancement of CN VII Spontaneous remission should begin within 3 wk of onset Paralysis or paresis of all muscle and VIII Delayed (3 to 6 mo) recovery portends at least some groups on one side of the face High resolution CT functional loss Absence of signs of CNS disease Px: Absence of signs of ear or CPA 90% recover spontaneously and completely overall; diseases >90% recovery if paralysis was incomplete Poorer if hyperacusis, >60 yr, diabetes, HTN, severe pain Ramsay-Hunt 4.5 to 9% of PFP Hx: Stapedial reflex absent Rx: Syndrome (Herpes Risk Factors: Hyperacusis Audiology – SNHL Pt. should avoid touching lesions to prevent spread of Zoster Oticus) >60 yr SNHL Viral ELISA studies to confirm infection Varicella zoster Impaired immunity Severe pain of pinna, mouth, or face MRI with gadolinium (86% of Systemic steroids can relieve pain, vertigo, avoid infection of Cancer P/E: facial nerves enhance) postherpetic neuralgia CN VII/VIII Radiotherapy Vesicles on pinna, ext. canal (errupt Acyclovir may lessen pain, aid healing of vesicles Chemotherapy 3-7 d after onset of pain) F/U: 2 to 4 wk Associated herpes zoster ophthalmicus Px: (uveitis, keratoconjunctivitis, optic Poorer prognosis than Bell’s palsy; 22% recover neuritis, or glaucoma) completely, 66% incomplete paralysis, 10% complete paralysis TEMPORAL BONE FRACTURE Longitudinal (90%) 20% have PFP Hx: Skull X-rays Px: Blow to side of head CT head Injury usually due to stretch or impingement; may P/E: recover with time Trauma to side of head Neuro findings consistent with epidural/subdural bleed Transverse (10%) 40% have PFP Hx: Skull X-rays Px: Blow to frontal or occipital area CT head Nerve transection more likely P/E: Trauma to front or back of head Iatrogenic Variable (depending on level of injury) Wait for lidocaine to wear off Rx: EMG Exploration if complete nerve paralysis No exploration if any movement present Source: Paul Warrick, MD Rhinitis

Definition • infammation of the lining (mucosa) of the nasal cavity Table 10. Classification of Rhinitis Inflammatory Non-Inflammatory • Perennial non-allergic • Rhinitis medicamentosa • Asthma, ASA sensitivity • Topical decongestants • Allergic • Hormonal Rhinitis medicamentosa: rebound • Seasonal • Pregnancy congestion due to the overuse of • Perennial • Estrogens intranasal vasoconstrictors. • Atrophic • Thyroid For prevention, use of these medications • Primary: Klebsiella ozena (especially in elderly) • Idiopathic vasomotor for only 5-7 d is recommended. • Acquired: post-surgery if too much mucosa or turbinate has been resected • Infectious • Viral: e.g. rhinovirus, influenza, parainfluenza, etc. • Bacterial: e.g. S. aureus • Fungal • Granulomatous: TB, syphilis, leprosy • Non-infectious • Sarcoidosis • Granulomatosis with polyangiitis • Irritant • Dust • Chemicals • Pollution OT23 Otolaryngology Rhinitis Toronto Notes 2014

Table 11. Nasal Discharge: Character and Associated Conditions Character Associated Conditions Watery/mucoid Allergic, viral, vasomotor, CSF leak (halo sign) Mucopurulent Bacterial, foreign body Serosanguinous Neoplasia Bloody Trauma, neoplasia, bleeding disorder, hypertension/vascular disease

Allergic Rhinitis (Hay Fever)

Definition • rhinitis characterized by an IgE-mediated hypersensitivity to foreign allergens • acute-and-seasonal or chronic-and-perennial • perennial allergic rhinitis ofen confused with recurrent colds

Etiology • when allergens contact the respiratory mucosa, specifc IgE antibody is produced in susceptible hosts • concentration of allergen in the ambient air correlates directly with the rhinitis symptoms Congestion reduces nasal airflow and allows the nose to repair itself (i.e. washes away the irritants). Epidemiology Treatment should focus on the initial • age at onset usually <20 yr insult rather than target this defense • more common in those with a personal or family history of allergies/atopy mechanism.

Clinical Features • nasal: obstruction with pruritus, sneezing • clear rhinorrhea (containing increased eosinophils) • itching of eyes with tearing • frontal headache and pressure • mucosa: swollen, pale, “boggy” • seasonal (summer, spring, early autumn) ƒ pollens from trees ƒ lasts several weeks, disappears and recurs following year at same time • perennial ƒ inhaled: house dust, wool, feathers, foods, tobacco, hair, mould ƒ ingested: wheat, eggs, milk, nuts ƒ occurs intermittently for years with no pattern or may be constantly present

Complications • chronic sinusitis/polyps • serous otitis media

Diagnosis • history • direct exam • allergy testing

Treatment • education: identifcation and avoidance of allergen • nasal irrigation with saline • antihistamines (e.g. diphenhydramine, fexofenadine) • oral decongestants (e.g. pseudoephedrine, phenylpropanolamine) • topical decongestant (may lead to rhinitis medicamentosa) • other topicals: steroids (futicasone), disodium cromoglycate, antihistamines, ipratropium bromide • oral steroids if severe • desensitization by allergen immunotherapy OT24 Otolaryngology Rhinitis/Rhinosinusitis Toronto Notes 2014

Vasomotor Rhinitis

• neurovascular disorder of nasal parasympathetic system (vidian nerve) afecting mucosal blood vessels • nonspecifc refex hypersensitivity of nasal mucosa • caused by: ƒ temperature change ƒ alcohol, dust, smoke ƒ stress, anxiety, neurosis ƒ endocrine: hypothyroidism, pregnancy, menopause ƒ parasympathomimetic drugs ƒ beware of rhinitis medicamentosa: reactive vasodilation due to prolonged use (>5 d) of nasal drops and sprays (Dristan®, Otrivin®)

Clinical Features • chronic intermittent nasal obstruction, varies from side to side • rhinorrhea: thin, watery • mucosa and turbinates: swollen • nasal allergy must be ruled out

Treatment • elimination of irritant factors • parasympathetic blocker (Atrovent® nasal spray) • steroids (e.g. beclomethasone, futicasone) • surgery (ofen of limited lasting beneft): electrocautery, cryosurgery, laser treatment or removal of inferior or middle turbinates • vidian neurectomy (rarely done) • symptomatic relief with exercise (increased sympathetic tone) Rhinosinusitis

Pathogenesis of Rhinosinusitis • ostial obstruction or dysfunctional cilia permit stagnant mucous and consequently infection • all sinuses drain to a common prechamber under the middle meatus called the osteomeatal complex

Definition • infammation of the mucosal lining of the sinuses and nasal passages

Classification • acute: <4 wk • subacute: 4-8 wk • chronic: >8-12 wk Table 12. Etiologies of Rhinosinusitis Ostial obstruction Inflammation • URTI • Allergy Mechanical • Septal deviation • Turbinate hypertrophy • Polyps • Tumours • Adenoid hypertrophy • Foreign body • Congenital abnormalities (e.g. cleft palate) Immune • Granulomatosis with polyangiitis • Lymphoma, leukemia • Immunosuppressed patients (e.g. neutropenics, diabetics, HIV) Systemic • Cystic fibrosis • Immotile cilia (e.g. Kartagener’s) Direct extension Dental • Infection Trauma • Facial fractures OT25 Otolaryngology Rhinosinusitis Toronto Notes 2014

Acute Bacterial Rhinosinusitis

Definition • bacterial infection of the paranasal sinuses and nasal passages lasting >7 d • clinical diagnosis requiring ≥2 major symptoms, at least one of the symptoms is either nasal obstruction or purulent/discoloured nasal discharge ƒ major symptoms ƒ minor symptoms Š facial pain/pressure/fullness Š headache Š nasal obstruction Š halitosis Š purulent/discoloured nasal discharge Š fatigue Š hyposmia/anosmia Š dental pain Š cough Š ear pain/fullness

Etiology • bacteria: S. pneumoniae (35%), H. infuenzae (35%), M. catarrhalis, S. aureus, anaerobes (dental) • children are more prone to a bacterial etiology, but viral is still more common Acute Rhinosinusitis Complications Consider hospitalization if any of the • maxillary sinus most commonly afected following are suspected • must rule out fungal causes (mucormycosis) in immunocompromised hosts (especially if • Orbital (Chandler’s classification) painless, black or pale mucosa on examination) • Periorbital cellulitis • Orbital cellulitis • Subperiosteal abscess Clinical Features • Orbital abscess • sudden onset of • Cavernous sinus thrombosis ƒ nasal blockage/congestion and/or purulent nasal discharge/posterior nasal drip • Intracranial ƒ ± facial pain or pressure, hyposmia, sore throat • Meningitis • Abscess • persistent/worsening symptoms >5 to 7 d or presence of purulence for 3 to 4 d with high fever • Bony • speculum exam: erythematous mucosa, mucopurulent discharge, pus originating from the • Subperiosteal frontal bone middle meatus abscess (“Pott’s Puffy tumour”) • predisposing factors: viral URTI, allergy, dental disease, anatomical defects • Osteomyelitis • Neurologic • diferentiate from acute viral rhinosinusitis (course: <10 d, peaks by 3 d) • Superior orbital fissure syndrome (CN III/IV/VI palsy, immobile Management globe, dilated pupils, ptosis, V1 • depends on symptom severity (i.e. intensity/duration of symptoms, impact on quality of life) hypoesthesia) • Orbital apex syndrome • mild-moderate: INCS (as above, plus neuritis, ƒ if no response within 72 h, add antibiotics papilledema, decreased visual • severe: INCS + antibiotics acuity) • antibiotics: ƒ 1st line: amoxicillin x 10 d (TMP-SMX or macrolide if penicillin allergy) • if no response to 1st line antibiotics within 72 h, switch to 2nd line ƒ 2nd line: fuoroquinolones or amoxicillin-clavulinic acid inhibitors • adjuvant therapy (saline irrigation, analgesics, oral/topical decongestant) may provide FESS = Functional Endoscopic Sinus Surgery symptomatic relief Opening of the entire osteomeatal • CT indicated only if complications are suspected complex in order to facilitate drainage while sparing the sinus mucosa. Chronic Rhinosinusitis

Definition • infammation of the mucosa of paranasal sinuses and nasal passages >8 to 12 wk • diagnosis requiring ≥2 major symptoms for >8 to 12 wk and ≥1 objective fnding of infammation of the paranasal sinuses (CT/endoscopy)

Etiology • unclear etiology but the following may contribute or predispose: ƒ inadequate treatment of acute rhinosinusitis ƒ bacterial colonization/bioflms Allergic fungal rhinosinusitis is a chronic Š S. aureus, enterobacteriaceae, pseudomonas, S. pneumoniae, H. infuenza, β-hemolytic sinusitis affecting mostly young, immunocompetent, atopic individuals. streptococci Treatment options include FESS ƒ fungal infection (e.g. Aspergillus, Zygomycetes, Candida) ± intranasal topical steroids, ƒ anatomic abnormality (e.g. lost ostia patency, deviated septum – predisposing factors) antifungals, and immunotherapy. ƒ allergy/allergic rhinitis ƒ ciliary disorder (e.g. cystic fbrosis, Kartenger’s) ƒ chronic infammatory disorder (e.g. granulomatosis with polyangiitis) ƒ untreated dental disease OT26 Otolaryngology Rhinosinusitis/Epistaxis Toronto Notes 2014

Clinical Features (similar to acute, but less severe) • chronic nasal obstruction • purulent anterior/posterior nasal discharge • facial congestion/fullness • facial pain/pressure • hyposmia/anosmia • halitosis • chronic cough • maxillary dental pain Management • identify and address contributing or predisposing factors • obtain CT or perform endoscopy • if polyps present: INCS, oral steroids ± antibiotics (if signs of infection), refer to Otolaryngologist/Head and Neck Surgeon • if polyps absent: INCS, antibiotics, saline irrigation, oral steroids (severe cases) • antibiotics for 3 to 6 wk ƒ amoxillin-clavulinic acid inhibitors, fuoroquinolone (moxifoxacin), macrolide (clarithromycin), clindamycin, Flagyl® (metronidazole) • surgery if medical therapy fails or fungal sinusitis: FESS, balloon sinoplasty Complications • same as acute sinusitis, mucocele Epistaxis

Blood Supply to the Nasal Septum (see Figure 4, OT3) 1. Superior posterior septum: ƒ internal carotid ophthalmic anterior/posterior ethmoidal 2. Posterior septum: ƒ external carotid internal maxillary sphenopalatine artery nasopalatine 3. Lower anterior septum: ƒ external carotid facial artery superior labial artery nasal branch ƒ external carotid internal maxillary descending palatine greater palatine 90% of nose bleeds occur in Little’s area. • these arteries all anastomose to form Kiesselbach’s plexus, located at Little’s area (anterior- inferior portion of the cartilaginous septum) • bleeding from above middle turbinate is internal carotid, and from below is external carotid Table 13. Etiology of Epistaxis Type Causes Local Trauma (most common) Tumours • Fractures: facial, nasal • Benign: polyps, inverting papilloma, angiofibroma • Self-induced: digital, foreign body • Malignant: squamous cell carcinoma, esthesioneuroblastoma Iatrogenic: nasal, sinus, orbit surgery Inflammation • Rhinitis: allergic, non-allergic Barometric changes • Infections: bacterial, viral, fungal Nasal dryness: dry air ± septal deformities Idiopathic Septal perforation Chemical: cocaine, nasal sprays, ammonia, etc. Systemic Coagulopathies Special Cases • Meds: anticoagulants, NSAIDs • Adolescent male with unilateral • Hemophilias, von Willebrand’s recurrent epistaxis – consider juvenile nasopharyngeal angiofibroma (JNA). • Hematological malignancies This is the most common benign • Liver failure, uremia tumour of the nasopharynx Vascular: hypertension, atherosclerosis, • Thrombocytopenic patients – use Osler-Weber-Rendu (hereditary hemorrhagic telangectasia) resorbable packs to avoid risk of re-bleeding caused by pulling out the Others: GPA, SLE removable pack

Investigations • CBC, PT/PTT (if indicated) • x-ray, CT as needed Treatment • locate bleeding and achieve hemostasis 1. ABCs • patient leans forward to minimize swallowing blood and avoid airway obstruction • apply constant frm pressure for 20 min on cartilaginous part of nose (not bony pyramid) • if signifcant bleeding, assess vitals for signs of hemorrhagic shock ± IV NS, cross match blood OT27 Otolaryngology Epistaxis/Hoarseness Toronto Notes 2014

2. Determine Site of Bleeding • anterior/posterior hemorrhage defned by location in relationship to bony septum • visualize nasal cavity with speculum • use cotton pledget with topical lidocaine ± topical decongestant (i.e. Otrivin®) to help identify area of bleeding (ofen anterior septum) • if suspicion of bleeding disorder, coagulation workup (platelet number and platelet function assay)

3. Control the Bleeding • frst line topical vasoconstrictors (Otrivin®) • if frst line fails and bleeding adequately visualized, cauterize with silver nitrate • do not cauterize both sides of the septum at one time due to risk of septal perforation from loss of septal blood supply A. Anterior hemorrhage treatment ƒ if fail to achieve hemostasis with cauterization: Š place anterior pack* with half inch Vaseline®-soaked ribbon gauze strips layered from nasal foor toward nasal roof extending to posterior choanae or lubricated absorbable packing (i.e. Gelfoam wrapped in Surgicel®) for 2 to 3 d Š can also attempt packing with Merocel® or nasal tampons of diferent shapes Š can also apply Floseal® (hemostatic matrix consisting of topical human thrombin and cross-linked gelatin) if other methods fail B. Posterior hemorrhage treatment ƒ if unable to visualize bleeding source, then usually posterior source: Š place posterior pack* using a Foley catheter, gauze pack, or Epistat® balloon Š subsequently, layer anterior packing bilaterally Š admit to hospital with packs in for 3 to 5 d Š watch for complications: hypoxemia (naso-pulmonic refex), toxic shock syndrome (Rx: remove packs immediately), pharyngeal fbrosis/stenosis, alar/septal necrosis, aspiration C. If anterior/posterior packs fail to control epistaxis ƒ ligation or embolization of culprit arterial supply by interventional radiology ƒ ± septoplasty

* antibiotics for any posterior pack or any pack lef for >48 h because of risk of toxic shock syndrome

4. Prevention • prevent drying of nasal mucosa with humidifers, saline spray, or topical ointments • avoidance of irritants • medical management of hypertension and coagulopathies Hoarseness

Definitions • hoarseness: change in voice quality, ranging from voice harshness to voice weakness. Refects abnormalities anywhere along the vocal tract from oral cavity to lungs If hoarseness present for >2 wk in a • dysphonia: a general alteration in voice quality smoker, laryngoscopy must be done to • aphonia: no sound emanates from vocal folds rule out malignancy.

Acute Laryngitis

Definition • <2 wk infammatory changes in laryngeal mucosa

Etiology Vocal Cord Paralysis • viral: infuenza, adenovirus Unilateral: affected cord lies in the • bacterial: Group A Streptococcus parmedian position, inadequate glottic • mechanical acute voice strain submucosal hemorrhage vocal cord edema hoarseness closure during phonation weak, • environmental: toxic fume inhalation breathy voice. Usually medializes with time whereby phonation and aspiration Clinical Features improve. Treatment options include voice therapy, injection laryngoplasty • URTI symptoms, hoarseness, aphonia, cough attacks, ± dyspnea (Radiesse), medialization using silastic • true vocal cords erythematous/edematous with vascular injection and normal mobility block. Treatment Bilateral: cords rest in midline therefore voice remains good but respiratory • usually self-limited, resolves within ~1 wk function is compromised and may • voice rest present as stridor. If no respiratory • humidifcation issues, may monitor closely and wait • hydration for improvement. If respiratory issues, intubate and will likely require a • avoid irritants (e.g. smoking) tracheotomy. • treat with antibiotics if there is evidence of coexistent bacterial pharyngitis OT28 Otolaryngology Hoarseness Toronto Notes 2014

Chronic Laryngitis

Definition • >2 wk infammatory changes in laryngeal mucosa

Etiology • repeated attacks of acute laryngitis • chronic irritants (dust, smoke, chemical fumes) • chronic voice strain • chronic rhinosinusitis with postnasal drip • chronic alcohol use • esophageal disorders: GERD, Zenker’s diverticulum, hiatus hernia • systemic: allergy, hypothyroidism, Addison’s disease

Clinical Features • chronic dysphonia: rule out malignancy • cough, globus sensation, frequent throat clearing 2º to GERD • laryngoscopy: cords erythematous, thickened with ulceration/granuloma formation, and normal mobility

Treatment • remove ofending irritants • treat related disorders (e.g. antisecretory therapy for GERD) • speech therapy with voice rest • ± antibiotics ± steroids to decrease infammation • laryngoscopy to rule out malignancy

Vocal Cord Polyps

Definition • structural manifestation of vocal cord irritation • acutely, polyp forms 2º to capillary damage in the subepithelial space during extreme voice exertion Vocal Cords: Polyps vs. Nodules Etiology Polyps Nodule • most common benign tumour of vocal cords Unilateral, Bilateral • voice strain (muscle tension dysphonia) asymmetric • laryngeal irritants (GERD, allergies, tobacco) Acute onset Gradual onset May resolve Often follow a spontaneously chronic course Epidemiology • 30 to 50 yr of age Subepithelial Acute: submucosal capillary hemorrhage or edema • M>F breakage Chronic: hyalinization within submucous Clinical Features lesion • hoarseness, aphonia, cough attacks ± dyspnea Soft, smooth, Acute: small, discrete • pedicled or sessile polyp on free edge of vocal cord fusiform, nodules pedunculated Chronic: hard, white, • typically polyp asymmetrical, sof, and smooth mass thickened fibrosed • more common on the anterior 1/3 of the vocal cord nodules • intermittent respiratory distress with large polyps Surgical Surgical excision if excision if refractory Treatment persistent or in presence of risk • avoid irritants factors for • endoscopic laryngeal microsurgical removal if persistent or if high risk of malignancy laryngeal cancer

Vocal Cord Nodules

Definition • vocal cord callus • aka “screamer’s or singer’s nodules”

Etiology • early nodules occur 2º to submucosal hemorrhage • mature nodules result from hyalinization which occurs with long term voice abuse • chronic voice strain • frequent URTI, smoke, alcohol OT29 Otolaryngology Hoarseness/Salivary Glands Toronto Notes 2014

Epidemiology • frequently in singers, children, bartenders, and school teachers • F>M

Clinical Features • hoarseness worst at end of day • on laryngoscopy: ƒ ofen bilateral ƒ at the junction of the anterior 1/3 and posterior 2/3 of the vocal cords – point of maximal cord vibration • chronic nodules may become fbrotic, hard, and white

Treatment • voice rest • hydration • speech therapy • avoid irritants • surgery rarely indicated for refractory nodules

Benign Laryngeal Papillomas

Etiology • HPV types 6, 11 • possible hormonal infuence, possibly acquired during delivery

Epidemiology • biphasic distribution: 1) birth to puberty (most common laryngeal tumour) and 2) adulthood

Clinical Features • hoarseness and airway obstruction • can seed into tracheobronchial tree • highly resistant to complete removal • some juvenile papillomas resolve spontaneously at puberty • may undergo malignant transformation • laryngoscopy shows wart-like lesions in supraglottic larynx and trachea

Treatment • microdebridement or CO2 laser • adjuvants under investigation: interferon, cidofovir, acyclovir • HPV vaccine may prevent/decrease the incidence but more research is needed

Laryngeal Carcinoma

• see Neoplasms of the Head and Neck, OT34 Salivary Glands

Sialadenitis

Definition • infammation of salivary glands

Etiology • viral most common (mumps) • bacterial causes: S. aureus, S. pneumoniae, H. infuenzae • obstructive vs. non-obstructive • obstructive infection involves salivary stasis and bacterial retrograde fow

Predisposing Factors • HIV • anorexia/bulimia • Sjögren’s syndrome • Cushing’s, hypothyroidism, DM • hepatic/renal failure • meds that increase stasis: diuretics, TCAs, -blockers, anticholinergics, antibiotics • sialolithiasis (can cause chronic sialadenitis) OT30 Otolaryngology Salivary Glands Toronto Notes 2014

Clinical Features • acute onset of pain and edema of parotid or submandibular gland that may lead to marked swelling • ± fever Mumps usually presents with bilateral • ± leukocytosis parotid enlargement ± sensorineural • ± suppurative drainage from punctum of the gland hearing loss ± orchitis.

Investigations • U/S imaging to diferentiate obstructive vs. non-obstructive sialadenitis

Treatment • bacterial: treat with cloxacillin ± abscess drainage, sialogogues • viral: no treatment

Sialolithiasis

Definition • ductal stone (mainly hydroxyapatite) in adults, sand/sludge in children, leading to chronic sialadenitis • 80% in submandibular gland, <20% in parotid gland, ~1% in sublingual gland

Risk Factors • any condition causing duct stenosis or a change in salivary secretions (e.g. dehydration, diabetes, EtOH, hypercalcemia, psychiatric medication)

Clinical Features • pain and tenderness over involved gland Bilateral enlargement of the parotid • intermittent swelling related to meals glands may be a manifestation of a systemic disease, such as Sjögren’s • digital palpation reveals presence of calculus or an eating disorder (i.e. anorexia, bulimia). Investigations • ultrasound ± sialogram

Treatment • may resolve spontaneously • encourage salivation to clear calculus • massage, analgesia, antibiotics, sialogogues (e.g. lemon wedges, sour lemon candies), warm compresses • remove calculi endoscopically, by dilating duct or orifce, or by excision through foor of the mouth • if calculus is within the gland parenchyma, the whole gland must be excised

Salivary Gland Neoplasms

Etiology • anatomic distribution ƒ parotid gland: 70-85% ƒ submandibular gland: 8-15% ƒ sublingual gland: 1% ƒ minor salivary glands, most concentrated in hard palate: 5-8% A mass sitting above an imaginary line • malignant (see Table 15, OT35 and Table 16, OT36) drawn between the mastoid process and angle of the mandible is a parotid • benign neoplasm until proven otherwise. ƒ benign mixed (pleomorphic adenoma): 80% ƒ Warthin’s tumour (5 to 10% bilateral, M>F): 10% ƒ cysts, lymph nodes and adenomas: 10% ƒ oncocytoma: <1%

Epidemiology • 3 to 6% of all head and neck neoplasms in adults • mean age at presentation: 55 to 65 • M=F OT31 Otolaryngology Salivary Glands/Neck Masses Toronto Notes 2014

Parotid Gland Neoplasms

Clinical Features • 80% benign (pleomorphic adenoma – most common), 20% malignant (mucoepidermoid – most common) • painless slow-growing mass • if bilateral, suggests benign process (Warthin’s tumour, Sjögren’s, bulimia, mumps) or possible lymphoma

Investigations • FNA biopsy • CT or MRI to determine extent of tumour DDx Parotid Tumour Treatment Benign • Pleomorphic adenoma • treatment of choice is surgery for all salivary gland neoplasms – benign and malignant • Warthin’s tumour (more common • pleomorphic adenomas are excised due to risk of malignant transformation (5% risk over in men) prolonged period of time) • Benign lymphoepithelial cysts • superfcial tumour Malignant ƒ superfcial parotidectomy above plane of CN VII ± radiation • Mucoepidermoid carcinoma ƒ incisional biopsy contraindicated • Adenoid cystic carcinoma • Acinic cell carcinoma • deep lesion ƒ near-total parotidectomy sparing as much of CN VII as possible ƒ if CN VII involved then it is removed and cable grafed • complications of parotid surgery ƒ hematoma, infection, salivary fstula, temporary facial paresis, Frey’s syndrome (gustatory Frey’s syndrome is a post-operative sweating) complication characterized by gustatory sweating. It is due to aberrant Prognosis innervation of cutaneous sweat glands by parasympathetic nerve fibres that are • benign: excellent, <5% of pleomorphic adenomas may recur divided during surgery. • malignant: dependent on stage and type of malignancy (see OT36) Neck Masses

Approach to a Neck Mass

• ensure that the neck mass is not a normal neck structure (hyoid, transverse process of C1 vertebra, prominent carotid bulb) Inflammatory vs. Neoplastic Neck • any neck mass persisting for more than 2 wk should be investigated for possible neoplastic Masses causes Inflammatory Neoplastic History Table 14. Acquired Causes of Neck Lumps According to Age Painful Y N Age (yr) Possible Causes of Neck Lump H&N infection Y N Fever Y N <20 1. Congenital 2. Inflammatory/Infectious 3. Neoplastic Weight loss N Y CA risk factors N Y 20-40 1. Inflammatory 2. Congenital 3. Neoplastic Age Younger Older >40 1. Neoplastic 2. Inflammatory 3. Congenital Physical Tender Y N Differential Diagnosis Rubbery Y Occ. Rock hard N Y • congenital Mobile Y ± fixed ƒ lateral (branchial clef cyst, lymphatic/venous/venolymphatic malformation) ƒ midline (thyroglossal duct cyst, dermoid cyst, laryngocele) • infectious/infammatory ƒ reactive lymphadenopathy (20 to tonsillitis, pharyngitis) ƒ infectious mononucleosis ƒ Kawasaki, Kikuchi, Kimura ƒ HIV ƒ salivary gland calculi, sialadenitis ƒ thyroiditis • granulomatous disease ƒ mycobacterial infections ƒ sarcoidosis • neoplastic ƒ lymphoma ƒ salivary gland tumours ƒ thyroid tumours ƒ metastatic malignancy ("unknown primary") OT32 Otolaryngology Neck Masses/Congenital Neck Masses Toronto Notes 2014

Evaluation

Investigations • history and physical (including nasopharynx and larynx) • all other investigations and imaging are dependent upon clinical suspicion following history and physical • laboratory investigations ƒ WBC: infection vs. lymphoma ƒ Mantoux TB test ƒ thyroid function tests and scan • imaging ƒ neck U/S ƒ CT scan ƒ angiography: vascularity and blood supply to mass ƒ radiologic exam of stomach, bowel and sinuses • biopsy: for histologic examination ƒ FNA: least invasive ƒ needle biopsy ƒ open biopsy: for lymphoma • identifcation of possible primary tumour (rule out a metastatic lymph node from an “unknown primary”) ƒ panendoscopy: nasopharyngoscopy, laryngoscopy, esophagoscopy, bronchoscopy with washings, and biopsy of suspicious lesions ƒ biopsy of normal tissue of nasopharynx, tonsils, base of tongue, and hypopharynx ƒ primary identifed 95% of time stage and treat ƒ primary occult 5% of time: excisional biopsy of node for histologic diagnosis manage with radiotherapy and/or neck dissection (squamous cell carcinoma) Congenital Neck Masses

Branchial Cleft Cysts/Fistula

Embryology • at the 6th week of development, the 2nd branchial arch grows over the 3rd and 4th arches and fuses with the neighbouring caudal pre-cardial swelling forming the cervical sinus • 3 types of malformations: 1. branchial fstula: persistent communication between skin and GI tract 2. branchial sinus: blind-ended tract opening to skin 3. branchial cyst: persistent cervical sinus with no external opening

Clinical Features • 2nd branchial clef malformations most common ƒ sinuses and fstulae present in infancy as a small opening anterior to the sternocleidomastoid muscle ƒ cysts present as a smooth, painless, slowly enlarging lateral neck mass, ofen following an URTI • 1st branchial clef malformations present as sinus/fstula or cyst in preauricular area or over angle of mandible • 3rd branchial clef malformations present as recurrent thyroiditis or thyroid abscess and have a tract leading usually to the lef pyriform sinus • there is controversy whether or not 4th branchial clef anomalies exist, as they may be remnants of the thyrothymic axis

Treatment • surgical removal of cyst or fstula tract • if infected: allow infection to settle before removal OT33 Otolaryngology Congenital Neck Masses Toronto Notes 2014

IX

External carotid a. XII Internal carotid a. IX

Middle constrictor m. XII Hyoid Type I anomaly Thyrohyoid membrane

Thyroid cartilage Type II anomaly

Common carotid a.

A. First Branchial Anomaly B. Second Branchial Anomaly XII

XII

Hyoid

XII Thyroid cartilage Cricoid cartilage Internal carotid a. XII External carotid a. Left common carotid a. Right common carotid a. Hyoid

Thyroid cartilage Left subclavian a. Cricoid cartilage

Brachiocephalic trunk

Arch of aorta

C. Third Branchial Anomaly D. Fourth Branchial Anomaly © M. Romanova 2010 © M. Romanova Figure 19. Branchial cleft cysts

Thyroglossal Duct Cysts

Embryology • thyroid originates as ventral midline diverticulum at base of tongue caudal to junction of 3rd and 4th branchial arches (foramen cecum) and migrates down to inferior aspect of neck • thyroglossal duct cysts are vestigial remnants of tract

Clinical Features • usually presents in childhood or 2nd to 4th decades as a midline cyst that enlarges with URTI and elevates with swallowing and tongue protrusion

Treatment • pre-operative antibiotics to reduce infammation • small potential for neoplastic transformation so complete excision of cyst and tissue around tract up to foramen cecum at base of tongue with removal of central portion of hyoid bone (Sistrunk procedure) recommended OT34 Otolaryngology Congenital Neck Masses/Neoplasms of the Head and Neck Toronto Notes 2014

Lymphatic Malformation

Definition • lymphatic malformation arising from vestigial lymph channels of neck

Clinical Features • usually present by age 2 • can be macrocystic (composed of large thin-walled cysts, usually below level of mylohyoid muscle) or microcystic (composed of minute cysts, usually above level of mylohyoid muscle) • usually painless, sof, compressible • infection causes a sudden increase in size

Treatment • can regress spontaneously afer bacterial infection, therefore do not plan surgical intervention until several months afer infection • macrocystic lesions can be treated by sclerotherapy or surgical excision • microcystic lesions are difcult to treat, but can be debulked Neoplasms of the Head and Neck

Pre-Malignant Disease • leukoplakia ƒ hyperkeratosis All patients presenting with a head ƒ risk of malignant transformation 5 to 20% and neck mass should be asked if • erythroplakia they are experiencing the following ƒ obstructive, referred, or local red superfcial patches adjacent to normal mucosa symptoms: ƒ commonly associated with epithelial dysplasia • Dyspnea or stridor ƒ associated with carcinoma in situ or invasive tumour in 40% of cases (positional vs. non-positional) • dysplasia • Hoarseness or dysphonia ƒ • Otalgia histopathologic presence of mitoses and prominent nucleoli • Non-healing oral ulcer ƒ involvement of entire mucosal thickness = carcinoma in situ • Dysphagia ƒ associated progression to invasive cancer in 15 to 30% of cases • Hemoptysis, hematemesis

Investigations • initial metastatic screen includes chest x-ray • scans of liver, brain, and bone only if clinically indicated Detection of cervical lymph nodes on • CT scan is superior to MRI for the detection of pathologic nodal disease and bone cortex invasion physical examination: • MRI is superior to discriminate tumour from mucus and to detect bone marrow invasion False negative rate: 15 to 30% • ± PET scans False positive rate: 30 to 40%

Treatment • treatment depends on: ƒ histologic grade of tumour ƒ stage Pathological lymphadenopathy defined radiographically as: ƒ physical and psychological health of patient • A jugulodigastric node >1.5 cm in ƒ facilities available diameter, or a retropharyngeal node ƒ expertise and experience of the medical and surgical oncology team >1 cm in diameter • in general: • A node of any size which contains central necrosis ƒ 1º surgery for malignant oral cavity tumours with radiotherapy reserved for salvage or poor prognostic indicators ƒ 1º radiotherapy for nasopharynx, oropharynx, hypopharynx, larynx malignancies with surgery reserved for salvage ƒ palliative chemotherapy for metastatic or incurable disease ƒ concomitant chemotherapy increases survival in advanced disease Common sites of distant metastases for ƒ chemotherapy has a role as induction therapy prior to surgery and radiation head and neck neoplasms: ƒ panendoscopy to detect primary disease when lymph node metastasis is identifed lungs > liver > bones ƒ anti-EGFR treatment (cetuximab, panitumumab) has a role as concurrent therapy with radiation (for advanced local and regional disease)

Prognosis • synchronous tumours occur in 9 to 15% of patients • late development of 2nd primary most common cause of post-treatment failure afer 36 mo OT35 Otolaryngology Neoplasms of the Head and Neck Toronto Notes 2014

Table 15. Quick Look-Up Summary of Head and Neck Malignancies – Etiology and Epidemiology Etiology Epidemiology Risk Factors Oral Cavity Risk Factors for Head and Neck 95% SCC Mean age: 50 to 60 Smoking/EtOH Cancer include: others: sarcoma, melanoma, M>F Poor oral hygiene • Smoking minor salivary gland tumour Most common site of H&N cancers Leukoplakia, erythroplakia • EtOH (this is synergistic with smoking) 50% on anterior 2/3 of tongue Lichen planus, chronic inflammation • Radiation Sun exposure – lip • Occupational/Environmental exposures • Oral HPV infection (independent of HPV infection smoking and EtOH exposure) Nose and Paranasal Sinus 75 to 80% SCC Mean age: 50 to 70 Wood/shoe/textile industry Adenocarcinoma (2nd most common) and Rare tumours Hardwood dust (nasal/ethmoid sinus) mucoepidermoid incidence in last 5 to 10 yr Nickel, chromium (maxillary sinus) 99% in maxillary/ethmoid sinus Air pollution 10% arise from minor salivary glands Chronic rhinosinusitis Carcinoma of the Pharynx – Subtypes (Nasopharynx, Oropharynx, Hypopharynx and Larynx) Nasopharynx 90% SCC Mean age: 50 to 59 Epstein-Barr virus (EBV) ~10% lymphoma M:F= 2.4:1 Salted fish Incidence 0.8 per 100,000 Nickel exposure 100x increased incidence in Southern Poor oral hygiene Chinese Genetic – Southern Chinese Oropharynx 95% SCC – poorly differentiated Mean age: 50 to 70 Smoking/EtOH M:F = 4:1 HPV Infection Hypopharynx 95% SCC Mean age: 50 to 70 Smoking/EtOH 3 sites: M>F 1. pyriform sinus (60%) 8 to 10% of all H&N cancer 2. post-cricoid (30%) 3. post pharyngeal wall (10%) Larynx SCC most common Mean age: 45 to 75 Smoking/EtOH 3 sites: M:F = 10:1 HPV 16 infection strongly associated 1. supraglottic (30 to 35%) 45% of all H&N cancer with the risk of laryngeal squamous cell 2. glottic (60 to 65%) cancers (Li et al., 2013) 3. subglottic (1%) Salivary Gland The smaller the salivary gland, the 40% mucoepidermoid Mean age: 55 to 65 greater the likelihood that a mass in the 30% adenoid cystic M=F gland is malignant. 5% acinic cell 3 to 6% of all H&N cancer 5% malignant mixed Rate of malignancy: 5% lymphoma Parotid 15 to 25% Submandibular 37 to 43% Minor salivary >80% Thyroid ( 90% benign – 10% malignant) >80% papillary Children Radiation exposure 5-15% follicular Adults <30 or >60 Family history – papillary CA or multiple 5% medullary Nodules more common in females endocrine neoplasia – MEN II <5% anaplastic Malignancy more common in males Older age 1 to 5% Hurthle cell Male 1 to 2% metastatic Papillary – Gardner’s, Cowden’s, familial adenomatous polyposis (FAP) Parathyroid Mean age: 44 to 55 yr Rare tumour OT36 Otolaryngology Neoplasms of the Head and Neck Toronto Notes 2014

Table 16. Quick Look-Up Summary of Head and Neck Malignancies – Diagnosis and Treatment Clinical Features Investigations Treatment Prognosis Oral Cavity Asymptomatic neck mass (30%) Biopsy 1o surgery 5 yr survival: - T1/T2: 75% Non-healing ulcer ± bleeding CT local resection - T3/T4: 30 to 35% Dysphagia, sialorrhea, dysphonia ± neck dissection Poor prognostic indicators: Oral fetor, otalgia, leukoplakia or ± reconstruction Depth of invasion, close surgical margins location (tongue erythroplakia (pre-malignant 2o radiation worse than floor of mouth) changes or CIS) Cervical nodes, extra capsular spread Nose and Paranasal Sinus Early symptoms: CT/MRI Surgery and radiation 5 yr survival: 30 to 60% Unilateral nasal obstruction Biopsy Chemoradiotherapy Poor prognosis 2o to late presentation Epistaxis, rhinorrhea Late symptoms: 2o to invasion of nose, orbit, nerves, oral cavity, skin, skull base, cribriform plate Nasopharynx Cervical nodes (60 to 90%) Nasopharyngoscopy 1o radiation, chemoradiation 5 yr survival: Nasal obstruction, epistaxis Biopsy Surgery for limited or recurrent disease - I: 79% Unilateral otitis media ± hearing loss CT/MRI - II: 72% CN III to VI, IX to XII (25%) - III: 50 to 60% Proptosis, voice change, dysphagia - IV: 36 to 42% Oropharynx Odynophagia, otalgia Biopsy 1o radiation Base of tongue – control rates Ulcerated/enlarged tonsil CT 2o surgery T1: >90%, T4: 13 to 52% Fixed tongue/trismus/dysarthria local resection Tonsils – cure rate Oral fetor, bloody sputum ±neck dissection T1/T2: 90 to 100%, T4: 15 to 33% Cervical lymphadenopathy (60%) ±reconstruction HPV-positive tumours have an approximately Distant mets: lung/bone/liver (7%) 20% improved overall survival rate Hypopharynx Dysphagia, odynophagia Pharyngoscopy 1o radiation 5 yr survival: Otalgia, hoarseness Biopsy 2o surgery T1: 53% Cervical lymphadenopathy CT T2/T3: 36-39% T4: 24% Larynx Dysphagia, odynophagia, globus Laryngoscopy 1o radiation 5 yr survival: T4 >40% (surgery with radiation) Otalgia, hoarseness, CT/MRI 2o surgery Control rate early lesions >90% (radiation) Dyspnea/stridor 1o surgery for bulky T4 disease 10 to 12% of small lesions fail radiotherapy Cough/hemoptysis Cervical nodes (rare w/ glottic CA) Salivary Gland Painless mass FNA 1º surgery ± neck dissection: Parotid: CN VII palsy MRI/CT Post-op radiotherapy 10 yr survival: 85, 69, 43, and 14% for stages I to IV Cervical lymphadenopathy Chemotherapy if unresectable Submandibular: Rapid growth 2 yr survival: 82%, 5 year: 69% Invasion of skin Minor salivary gland: Constitutional signs/symptoms 10 yr survival: 83, 52, 25, 23% for stages I to IV Thyroid Thyroid mass, cervical nodes FNA 1o surgery Recurrences occur within 5 yr Vocal cord paralysis U/S I131 for intermediate and high risk well Need long-term f/u: clinical exam, thyroglobulin Hyper/hypothyroidism differentiated thyroid cancer Dysphagia Parathyroid Increased serum Ca2+ Sestamibi Wide surgical excision Recurrence rates Neck mass Post-op monitoring of serum Ca2+ 1 yr: 27% Bone disease, renal disease 5 yr: 82% Pancreatitis 10 yr: 91% Mean survival: 6 to 7 yr OT37 Otolaryngology Neoplasms of the Head and Neck Toronto Notes 2014

Thyroid Carcinoma

Table 17. Bethesda Classification of Thyroid Cytology Category Risk of Malignancy Non-diagnostic or unsatisfactory Unknown Benign 0-3% Follicular lesion of undetermined significance/ 5-15% Atypia of undetermined significance Follicular/Hurthle cell neoplasms 15-30% Suspicious for malignancy 60-75% Malignant 97-99%

Table 18. Thyroid Carcinoma Papillary Follicular Medullary Anaplastic Lymphoma Incidence 70 to 75% 10% 3 to 5% <5% <1% (% of all thyroid (10% familial cancers) 90% sporadic) Route of Lymphatic Hematogenous Lymphatic and hematogenous Spread Histology Orphan Annie nuclei Capsular/vascular invasion Amyloid Giant cells Psammoma bodies Invasion influences May secrete calcitonin, Spindle cells Papillary architecture prognosis prostaglandins, ACTH, serotonin, kallikrein or bradykinin Other Ps – Papillary cancer Fs – Follicular cancer Ms – Medullary cancer More common in elderly Usually non-Hodgkin’s lymphoma Popular (most common) Far away mets Multiple endocrine 70% in women Rapidly enlarging thyroid mass Palpable lymph nodes Female (3:1) neoplasia (MEN IIa or IIb) 20 to 30% have Hx of Hx of Hashimoto’s thyroiditis Positive I131 uptake NOT FNA (can’t be aMyloid differentiated thyroid Ca increases risk 60x Positive prognosis diagnosed by FNA) Median node dissection (mostly papillary) 4:1 female predominance Post-op I131 scan to guide Favourable prognosis or nodular goiter mass dysphagia, dyspnea, stridor, treatments Rapidly enlarging neck hoarseness, neck pain, facial Rule out lymphoma edema, accompanied by “B” symptoms* Prognosis 98% at 10 yr 92% at 10 yr 50% at 10 yr 20 to 35% at 1 yr 5 yr survival 20% at 10 yr if detected 13% at 10 yr Stage IE 55%-80% when clinically palpable Stage IIE 20%-50% Stage IIE/IV 15%-35% Treatment Small tumours: Small tumours: Total thyroidectomy Radiation and chemotherapy Non-surgical Near total Near total thyroidectomy/ Median lymph node dissection Small tumours: Combined radiation thyroidectomy or lobectomy lobectomy/isthmectomy if lateral cervical nodes +ve Total thyroidectomy Chemotherapy (CHOP**) Diffuse/bilateral: Large/diffuse tumours: Modified neck dissection ± external beam Total thyroidectomy Total thyroidectomy Post-op thyroxine ± Post-op I131 tx Tracheostomy Screen asymptomatic relatives *B symptoms = fever, night sweats, weight loss >10% in 6 mo ** CHOP = cyclophosphamide, adriamycin, vincristine, prednisone

Approach to Thyroid Nodule • all patients with thyroid nodules require evaluation of serum TSH and ultrasound • any nodule >5 mm with suspicious sonographic features (particularly microcalcifcations) Indications for post-op radioactive should undergo FNA iodine ablation – I131 • any nodule >1 cm should undergo FNA Adjuvant therapy: decrease recurrent • when performing repeat FNA on initially non-diagnostic nodules, U/S-guided FNA should be disease employed RAI therapy: treat persistent cancer • nuclear scanning has minimal value in the investigation of the thyroid nodule

Table 19. Management of the Thyroid Nodule Treatment Indications Radioiodine therapy For the treatment of hyperthyroidism or as adjuvant treatment after surgery in the treatment of papillary or follicular carcinoma Chemotherapy and/or radiotherapy Anaplastic CA or thyroid lymphoma Surgical excision Mass that is “suspicious” on FNA Malignancy other than anaplastic CA or thyroid lymphoma Mass that on FNA is benign but increasing in size on serial imaging and/or >3-4 cm in size Hyperthyroidism not amenable to medical therapy *U/S findings: cystic: risk of malignancy <1%, solid: risk of malignancy approx. 10%, solid with cystic components: risk of malignancy same as if solid OT38 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Pediatric Otolaryngology

Acute Otitis Media (AOM)

Definition • acute infammation of middle ear

Epidemiology • 60 to 70% of children have at least 1 episode of AOM before 3 yr of age • 18 mo to 6 yr most common age group • peak incidence January to April • one third of children have had ≥3 episodes by age 3

Etiology • S. pneumoniae: 35% of cases (incidence decreasing due to pneumococcus vaccine) • H. infuenzae: 25% of cases • M. catarrhalis: 10% of cases • S. aureus and S. pyogenes (all -lactamase producing) • anaerobes (newborns) • Gram-negative enterics (infants) • viral

Predisposing Factors • Eustachian tube dysfunction/obstruction: ƒ swelling of tubal mucosa: Š upper respiratory tract infection (URTI) Š allergic rhinitis Š chronic rhinosinusitis ƒ obstruction/infltration of Eustachian tube ostium: Š tumour: nasopharyngeal carcinoma (adults) Š adenoid hypertrophy (not due to obstruction but by maintaining a source of infection) Š barotrauma (sudden changes in air pressure) ƒ inadequate tensor palati function: clef palate (even afer repair) ƒ abnormal Eustachian tube: Š Down syndrome (horizontal position of Eustachian tube), Crouzon syndrome, and Apert syndrome • disruption of action of: ƒ cilia of Eustachian tube: Kartagener's syndrome ƒ mucus secreting cells ƒ capillary network that provides humoral factors, PMNs, phagocytic cells • immunosuppression/defciency due to chemotherapy, steroids, diabetes mellitus, hypogammaglobulinemia, cystic fbrosis

Risk Factors • bottle feeding, pacifer use • second-hand smoke • crowded living conditions (day care/group child care facilities) or sick contacts • male • family history

Pathogenesis • obstruction of Eustachian tube air absorbed in middle ear negative pressure (an irritant to middle ear mucosa) edema of mucosa with exudate/efusion infection of exudate from nasopharyngeal secretions

Clinical Features • triad of otalgia, fever (especially in younger children), and conductive hearing loss • rarely tinnitus, vertigo, and/or facial nerve paralysis • otorrhea if tympanic membrane perforated Clinical Assessment of AOM in Paediatrics • infants/toddlers JAMA 2010;304:2161-2169 ƒ ear-tugging (this alone is not a good indicator of pathology) ƒ hearing loss, balance disturbances (rare) In assessment of AOM in paediatrics, ear pain is the most useful symptom with a likelihood ratio ƒ irritable, poor sleeping (LR) between 3.0 and 7.3. Useful otoscopic signs ƒ vomiting and diarrhea include erythematous (LR 8.4, 95% CI 7-11), cloudy ƒ anorexia (LR 34, 95% CI 28-42), bulging (LR 51, 95%CI • otoscopy of TM 36-73), and immobile tympanic membrane on ƒ hyperemia pneumatic otoscopy (LR 31, 95% CI 26-37). ƒ bulging, pus may be seen behind TM ƒ loss of landmarks: handle and long process of malleus not visible OT39 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Diagnosis and Management • American Academy of Pediatrics (AAP) Guidelines 2013 suggest the following action statements (adapted): Antibiotics for Acute Otitis Media in Children ƒ Diagnose AOM if: Cochrane DB Syst Rev 2004;1:CDOOO219 1. Moderate to severe bulging of TM or new onset of otorrhea not due to otitis externa Study: Meta-analysis of Randomized Controlled 2. Mild bulging of tympanic membrane and recent (<48 h) ear pain or intense erythema of TM Trials (RCTs) on children (>6 mo) with acute otitis media comparing any antibiotic regime to placebo. 3. Do not diagnose AOM if no middle ear efusion (based on pneumatic otoscopy or Data Sources: Cochrane Central Register of tympanometry) Controlled Trials (2003 issue 1), MEDLINE (January 2000 to March 2003), and EMBASE (January 1990 Management of AOM to March 2003) without language restrictions. Main Outcomes: 1) Pain at 24 h, and 2-7 d. 1. Assess for pain. If pain present, treat the pain 2) Hearing measured by tympanometry at 1 and 2. For severe unilateral or bilateral AOM (moderate or severe otalgia, or otalgia for 48 h, or 3 mo. temperature 39°), prescribe antibiotics if 6 mo or older Patients: Pain: 24 h, 4 studies (n=717); 2-7 d 3. For nonsevere bilateral AOM (mild otalgia, otalgia <48 h, temperature <39°), prescribe 9 studies (n=2287). Hearing: 1 mo, 3 studies antibiotics if 6 to 23 mo (n=472); 3 mo, 2 studies (n=370). Results: Treatment with antibiotics had no 4. For nonsevere unilateral AOM, prescribe antibiotics or observe with close follow up based on significant impact on pain at 24 h. However, pain joint decision making with parents if 6 to 23 mo at 2-7 d was lower in the antibiotic groups with 5. For nonsevere unilateral or bilateral AOM, prescribe antibiotics or observe with close follow an NNT of 16 (p<0.00001). Antibiotics had no up based on joint decision making with parents if 24 mo or older significant effect on hearing. 6. Antibiotic treatment when given should consist of: Conclusion: The role of antibiotics is largely ƒ restricted to pain control. This can also be achieved amoxicillin if child has not received amoxicillin in past 30 d, child does not have purulent by analgesics. Therefore, parents should be conjunctivitis, or child is not allergic to penicillin counseled that other analgesics may be a safer ƒ add β-lactamase coverage if received amoxicillin in past 30 d, has purulent conjunctivitis, option. or has history of recurrent AOM not responsive to amoxicillin ƒ reassess if symptoms worsen or fail to respond to treatment within 48 to 72 h ƒ do NOT prescribe prophylactic antibiotics to reduce frequency of AOM 7. Tympanostomy tubes can be ofered for recurrent AOM (3 episodes in 6 mo, 4 episodes in 1 yr) with 1 episode in preceding 6 mo 8. Recommend pneumococcal vaccine and annual infuenza vaccine to all children Indications for Myringotomy and Tympanostomy Tubes in Recurrent 9. Encourage exclusive breastfeeding for at least 6 mo AOM (RAOM) and OME 10. Avoid tobacco smoke • Persistent OME >3 mo • Lack of response to >3 mo of • antibiotic treatment hastens resolution: 10 d course antibiotic therapy ƒ 1st line: • Persistent effusion for ≥3 mo after Š episode of AOM amoxicillin 80-90 mg/kg/d divided into two doses: safe, efective, and inexpensive • RAOM (>3 episodes in 6 mo, or >4 Š if penicillin allergic: macrolide (clarithromycin, azithromycin – high resistance), in 12 mo) trimethoprim-sulphamethoxazole (Bactrim®) • Bilateral conductive hearing loss of ƒ 2nd line: >30 dB Š amoxicillin-clavulanic acid (Clavulin®) • Chronic retraction of the tympanic membrane or pars flaccida Š cephalosporins: cefuroxime axetil (Cefin®), cefriaxone (Rocephin®), cefaclor (Ceclor®), • Bilateral OME lasting >4 to 6 mo cefxime (Suprax®) • Craniofacial anomalies predisposing Š AOM deemed unresponsive if clinical signs/symptoms and otoscopic fndings persist to middle ear infections (e.g. cleft beyond 48 h of antibiotic treatment palate) • symptomatic therapy: • Complications of AOM or OME ƒ antipyretics/analgesics (e.g. acetaminophen) Otolaryngologists’ perceptions of the indications for ƒ decongestants: may relieve nasal congestion but does not treat AOM tympanostomy tube insertion in children. CMAJ 2000;162:1285-1288 • prevention: Clinical indicators myringotomy and tympanostomy ƒ tubes. American Academy of Otolaryngology – parent education about risk factors Head and Neck Surgery, 2010. Available at: http:// ƒ antibiotic prophylaxis: amoxicillin or macrolide shown efective at half therapeutic dose www.entnet.org/Practice/Myringotomy-and- ƒ pneumococcal and infuenza vaccine Tympanostomy-tubes.cfm ƒ surgery: Š choice of surgical therapy for recurrent AOM depends on whether local factors (Eustachian tube dysfunction) are responsible (use ventilation tubes), or regional disease factors (tonsillitis, adenoid hypertrophy, sinusitis) are responsible

Complications of AOM • otologic: ƒ TM perforation Complications of Tympanostomy Tubes ƒ chronic suppurative OM Early ƒ ossicular necrosis • Extrusion • Blockage ƒ cholesteatoma • Persistent otorrhea ƒ persistent efusion (ofen leading to hearing loss) Late • CNS: • Myringosclerosis ƒ meningitis • Persistent TM perforation ƒ brain abscess • Cholesteatoma ƒ facial nerve paralysis • other: ƒ mastoiditis ƒ labyrinthitis ƒ sigmoid sinus thrombophlebitis OT40 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Otitis Media with Effusion (OME)

Definition • presence of fuid in the middle ear without signs or symptoms of ear infection

Epidemiology • most common cause of pediatric hearing loss • not exclusively a pediatric disease • follows AOM frequently in children • middle ear efusions have been shown to persist following an episode of AOM for 1 mo in 40% of children, 2 mo in 20%, and 3+ mo in 10%

Risk Factors • same as AOM

Clinical Features • hearing loss ± tinnitus ƒ confrm with audiogram and tympanogram (fat) (see Figure 16B, OT10 and Figure 17B, OT11) • fullness – blocked ear • ± pain, low grade fever • otoscopy of tympanic membrane: ƒ discolouration – amber or dull grey with “glue” ear ƒ meniscus fuid level behind TM ƒ air bubbles ƒ retraction pockets/TM atelectasis ƒ most reliable fnding with pneumotoscopy is immobility

Treatment • expectant: 90% resolve by 3 mo • document hearing loss with audiogram • no statistical proof that antihistamines, decongestants, antibiotics clear disease faster • surgery: myringotomy ± ventilation tubes ± adenoidectomy (if enlarged or on insertion of second set of tubes afer frst set falls out) • ventilation tubes to equalize pressure and drain ear

Complications of Otitis Media with Effusion (OME) • hearing loss, speech delay, learning problems in young children • chronic mastoiditis • ossicular erosion • cholesteatoma especially when retraction pockets involve pars faccida or postero-superior TM • retraction of tympanic membrane, atelectasis, ossicular fxation

Adenoid Hypertrophy

• size peaks at age 5 and resolves by age 12 Pharyngeal tonsil • increase in size with repeated URTI and allergies (adenoid) Upper midline in nasopharynx Clinical Features • nasal obstruction: ƒ adenoid facies (open mouth, high arched palate, narrow midface, malocclusion) Tubal tonsil (x2) ƒ history of hypernasal voice and snoring Around openings ƒ long term mouth breather; minimal air escape through nose of eustachian tubes • choanal obstruction: Palatine tonsil (x2) ƒ chronic rhinosinusitis/rhinitis Either side ƒ obstructive sleep apnea of oropharynx • chronic infammation: Lingual tonsil ƒ nasal discharge, post-nasal drip, and cough Under mucosa of

posterior 1/3 of tongue © June Li 2010 ƒ cervical lymphadenopathy Figure 20. Waldeyer’s ring Diagnosis An interrupted circle of protective lymphoid • enlarged adenoids on nasopharyngeal exam (usually with fexible nasopharyngoscope) tissue at the upper ends of the respiratory • enlarged adenoid shadow on lateral sof tissue x-ray and alimentary tracts

Complications • Eustachian tube obstruction leading to serous otitis media • interference with nasal breathing, necessitating mouth-breathing • malocclusion • sleep apnea/respiratory disturbance • orofacial developmental abnormalities OT41 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Adenoidectomy

Indications for Adenoidectomy • chronic upper airway obstruction with sleep disturbance/apnea ± cor pulmonale • chronic nasopharyngitis resistant to medical treatment • chronic serous otitis media and chronic suppurative otitis media (with 2nd set of tubes) • recurrent acute otitis media resistant to antibiotics • suspicion of nasopharyngeal malignancy • persistent rhinorrhea

Contraindications • uncontrollable coagulopathy • recent pharyngeal infection • short or abnormal palate (clef or false palate, zona pellucida)

Complications • bleeding, infection • velopharyngeal insufciency (hypernasal voice or nasal regurgitation) • scarring of Eustachian tube orifce

Sleep-Disordered Breathing in Children

Definition • spectrum of sleep-related breathing abnormalities ranging from snoring to OSA

Epidemiology • peak incidence between 2 and 8 yr when tonsils and adenoids are the largest relative to the pharyngeal airway

Etiology • due to a combination of anatomic and neuromuscular factors: ƒ adenotonsillar hypertrophy ƒ craniofacial abnormalities ƒ neuromuscular hypotonia (i.e. cerebral palsy, Down syndrome) ƒ obesity

Clinical Features • heavy snoring, mouth breathing, pauses or apnea, enuresis, excessive daytime sleepiness, behavioural/learning problems, diagnosis of ADHD, morning headache, failure to thrive

Investigations • fexible nasopharyngoscopy for assessment of nasopharynx and adenoids • polysomnography (obstructive apnea-hypopnea index >1/h considered abnormal)

Treatment • surgical: bilateral tonsillectomy and adenoidectomy • nonsurgical: CPAP, BiPAP, sleep hygiene

Acute Tonsillitis

Etiology • Group A -hemolytic streptococci (most common) and Group C or G streptococci Trismus: motor disturbance of the • S. pneumoniae, S. aureus, H. infuenzae, M. catarrhalis trigeminal nerve, leading to spasm of the • EBV muscles of mastication, with difficulty in opening the mouth (lockjaw). Clinical Features • symptoms: ƒ sore throat ƒ dysphagia, odynophagia, trismus DDx Sore Throat ƒ malaise, fever • Streptococcal pharyngitis ƒ otalgia (referred) • Viral pharyngitis • signs: • Infectious mononucleosis ƒ tender cervical lymphadenopathy, especially submandibular, jugulodigastric • Tonsilitis • Peritonsillar abscess ƒ tonsils enlarged, infammation ± exudates/white follicles • Foreign body/trauma ƒ strawberry tongue, scarlatiniform rash (scarlet fever) • Leukemia ƒ palatal petechiae (infectious mononucleosis) • Hodgkin’s disease OT42 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Investigations • CBC • swab for C&S Complications of Tonsillitis • latex agglutination tests • Rheumatic heart disease • Monospot® – less reliable in children <2 yr old • Arthritis • Scarlet fever Treatment • Peritonsillar abscess (Quinsy), intratonsillar • sof diet, ample fuid intake • Deep neck space infection • gargle with warm saline solution • Sepsis • analgesics and antipyretics • Glomerulonephritis • antibiotics: ƒ only afer appropriate swab for C&S ƒ 1st line penicillin or amoxicillin (erythromycin if penicillin allergy) x 10 d ƒ rheumatic fever risk emerges approximately 9 d afer the onset of symptoms: Š antibiotics are utilized mainly to avoid this serious sequela and to provide earlier symptomatic relief ƒ no evidence for the role of antibiotics in the avoidance of post-streptococcal glomerulonephritis

Peritonsillar Abscess (Quinsy)

Definition • cellulitis of space behind tonsillar capsule extending onto sof palate leading to abscess

Etiology • bacterial: Group A strep (GAS) (50% of cases), S. pyogenes, S. aureus, H. infuenzae, and anaerobes

Epidemiology • can develop from acute tonsillitis with infection spreading into plane of tonsillar bed • unilateral • most common in 15 to 30 yr old age group Quinsy Triad Clinical Features • Trismus • fever and dehydration • Uvular deviation • sore throat, dysphagia, and odynophagia • Dysphonia ("hot potato voice") • extensive peritonsillar swelling but tonsil may appear normal • edema of sof palate • uvular deviation • involvement of motor branch of CN V (can lead to trismus) • dysphonia (edema failure to elevate palate) 2º to CN X involvement • unilateral referred otalgia • cervical lymphadenitis

Complications • aspiration pneumonia 2º to spontaneous rupture of abscess • airway obstruction • lateral dissection into parapharyngeal and/or carotid space • bacteremia • retropharyngeal abscess

Treatment • secure airway • surgical drainage (incision or needle aspiration) with C&S • warm saline irrigation • IV penicillin G x 10 d if cultures positive for GAS • add PO/IV metronidazole or clindamycin x 10 d if culture positive for Bacteroides • consider tonsillectomy afer second episode

Other Parapharyngeal Space Infection • pharyngitis • parotitis (see Salivary Glands, OT29) • otitis • mastoiditis (Bezold’s abscess) • odontogenic infection OT43 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Tonsillectomy

Absolute Indications • most common indication: sleep-disordered breathing • 2nd most common indication: recurrent throat infections • tonsillar hypertrophy causing upper airway obstruction, obstructive sleep apnea, severe dysphagia, or cardiopulmonary complications such as cor pulmonale • suspicion of malignancy (e.g. lymphoma, squamous cell carcinoma) • orofacial/dental deformity • hemorrhagic tonsillitis

Relative Indications (to reduce disease burden) • recurrent throat infection with a frequency of at least 7 episodes in the past year, at least 5 episodes per year for 2 yr, or at least 3 episodes per year for 3 yr, with documentation in the medical record for each episode of sore throat and 1 or more of the following: temperature >38.3°C, cervical adenopathy, tonsillar exudate, or positive test for Group A β-hemolytic streptococcus (Paradise Criteria) • chronic tonsillitis with halitosis (bad breath) or sore throat ± tonsilloliths (clusters of calcifed material that form in the crevices of the tonsils) • complications of tonsillitis: quinsy/peritonsillar abscess, parapharyngeal abscess, retropharyngeal abscess • failure to thrive

Relative Contraindications • velopharyngeal insufciency: overt or submucous/covert clef of palate, impaired palatal function due to neurological or neuro-muscular abnormalities • hematologic: coagulopathy, anemia • infectious: active local infection without urgent obstructive symptoms

Complications • hemorrhage: early – within 24 h; delayed – 7-10 d • odynophagia and/or otalgia; dehydration 20 to odynophagia • infection • atlantoaxial subluxation (Grisel’s syndrome): rare

Airway Problems in Children

DIFFERENTIAL DIAGNOSIS BY AGE GROUP

Neonates (obligate nose breathers) • extralaryngeal: ƒ choanal atresia (e.g. CHARGE syndrome) ƒ nasopharyngeal dermoid, glioma, encephalocele ƒ glossoptosis: Pierre-Robin sequence, Down syndrome, lymphatic malformation, hemangioma • laryngeal: ƒ laryngomalacia: most common cause of stridor in children ƒ laryngocele ƒ vocal cord palsy (due to trauma or Arnold-Chiari malformation) ƒ glottic web ƒ subglottic stenosis ƒ laryngeal clef • tracheal: ƒ tracheoesophageal fstula ƒ tracheomalacia ƒ vascular rings

2 to 3 Months • congenital: ƒ laryngomalacia ƒ vascular: subglottic hemangioma (more common), innominate artery compression, double aortic arch ƒ laryngeal papilloma • acquired: ƒ subglottic stenosis: post intubation ƒ tracheal granulation: post intubation ƒ tracheomalacia: post tracheotomy and TEF repair OT44 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Infants – Sudden Onset • foreign body aspiration • croup • bacterial tracheitis • caustic ingestion • epiglottitis

Children and Adults • infection: ƒ Ludwig’s angina ƒ peritonsillar/parapharyngeal abscess ƒ retropharyngeal abscess • neoplastic: ƒ squamous cell carcinoma (SCC) (adults): larynx, hypopharynx ƒ retropharyngeal: lymphoma, neuroblastoma ƒ nasopharyngeal: carcinoma, rhabdomyosarcoma • allergic: ƒ angioneurotic edema ƒ polyps (suspect cystic fbrosis in children) • trauma: ƒ laryngeal fracture, facial fracture ƒ burns and lacerations ƒ post-intubation ƒ caustic ingestion • congenital: ƒ lingual thyroid/tonsil

Signs of Airway Obstruction

Stridor • note quality, timing (inspiratory or expiratory) • body position important: ƒ lying prone: subglottic hemangioma, double aortic arch ƒ lying supine: laryngomalacia, glossoptosis • site of stenosis: ƒ vocal cords or above: inspiratory stridor ƒ subglottis and extrathoracic trachea: biphasic stridor ƒ distal tracheobronchial tree: expiratory stridor

Respiratory Distress • nasal faring • supraclavicular and intercostal indrawing • sternal retractions • use of accessory muscles of respiration • tachypnea • cyanosis • altered LOC

Feeding Difficulty and Aspiration • supraglottic lesion • laryngomalacia • vocal cord paralysis • laryngeal clef g aspiration pneumonia • TEF

Acute Laryngotracheobronchitis (Croup)

• infammation of tissues in subglottic space ± tracheobronchial tree • swelling of mucosal lining and associated with thick, viscous, mucopurulent exudate which Signs of Croup compromises upper airway (subglottic space narrowest portion of upper airway) The 3 Ss • normal function of ciliated mucous membrane impaired Stridor Subglottic swelling Etiology Seal bark cough • viral: parainfuenzae I (most common), II, III, infuenza A and B, RSV OT45 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Clinical Features • age: 4 mo to 5 yr • preceded by URTI symptoms • generally occurs at night • biphasic stridor and croupy cough (loud, sea-lion bark) • appear less toxic than epiglottitis • supraglottic area normal • rule out foreign body and subglottic stenosis • “steeple-sign” on AP x-ray of neck • if recurrent croup, think subglottic stenosis Treatment • racemic epinephrine via nebulizer q1-2h, prn (only if in respiratory distress) • systemic corticosteroids (e.g. dexamethasone, prednisone) • adequate hydration • close observation for 3 to 4 h • intubation if severe • hospitalize if poor response to steroids afer 4 h and persistent stridor at rest • consider alternate diagnosis if poor response to therapy (e.g. bacterial tracheitis) • if recurrent episodes of croup-like symptoms, consider bronchoscopy several weeks afer acute episode settles to rule out underlying subglottic stenosis Acute Epiglottitis

• acute infammation causing swelling of supraglottic structures of the larynx without involvement of vocal cords Etiology Acute epiglottitis is a medical • H. infuenzae type B emergency. • relatively uncommon condition due to Hib vaccine Clinical Features • any age, most commonly 1 to 4 yr • rapid onset • toxic-looking, fever, anorexia, restlessness • cyanotic/pale, inspiratory stridor, slow breathing, lungs clear with decreased air entry • prefers sitting up, open mouth, drooling, tongue protruding, sore throat, dysphagia Investigations and Management • investigations and physical examination may lead to complete obstruction, thus preparations for intubation or tracheotomy must be made prior to any manipulation • stat ENT/anesthesia consult(s) When managing epiglottitis, it is important not to agitate the child, as this • WBC (elevated), blood and pharyngeal cultures afer intubation may precipitate complete obstruction. • lateral neck radiograph (only done if patient stable) Treatment • secure airway • IV access with hydration Thumb sign: cherry-shaped epiglottic • antibiotics: IV cefuroxime, cefotaxime, or cefriaxone swelling seen on lateral neck radiograph. • moist air • extubate when leak around tube occurs and afebrile • watch for meningitis Subglottic Stenosis

Congenital • diameter of subglottis <4 mm in neonate (due to thickening of sof tissue of subglottic space or maldevelopment of cricoid cartilage) Acquired • following prolonged, repeated or traumatic intubation: ƒ most commonly due to endotracheal intubation; nasal intubation is less traumatic and preferred in long term intubation as it puts less pressure on the subglottis (tube sits at diferent orientation) and there is less movement ƒ subglottic stenosis is related to duration of intubation and pressure of the endotracheal tube cuf • can also be due to foreign body, infection (e.g. TB, diphtheria, syphilis) or chemical irritation Clinical Features • biphasic stridor • respiratory distress • recurrent/prolonged croup Diagnosis • rigid laryngoscopy and bronchoscopy OT46 Otolaryngology Pediatric Otolaryngology Toronto Notes 2014

Treatment • if sof stenosis: divide tissue with knife or laser, dilate with balloon ± steroids • if frm stenosis: laryngotracheoplasty Laryngomalacia

• short aryepiglottic folds, omega-shaped epiglottis, pendulous mucosa • caused by indrawing of supraglottis on inspiration leading to laryngopharyngeal refux of acid Clinical Features • high-pitched inspiratory stridor at 1 to 2 wk • constant or intermittent and more pronounced supine • usually mild but when severe can be associated with cyanosis or feeding difculties, leading to Laryngomalacia is the most common failure to thrive cause of stridor in infants. Treatment • observation is usually sufcient as symptoms spontaneously subside by 12 to 18 mo in >90% of cases • in the case of severe laryngomalacia, division of the aryepiglottic folds (supraglottoplasty) provides relief Foreign Body

Ingested • usually stuck at cricopharyngeus • coins, toys, batteries (emergency) • presents with drooling, dysphagia, stridor if very large Foreign body inhalation is the most Aspirated common cause of accidental death in • usually stuck at right mainstem bronchus children. • peanuts, carrot, apple core, popcorn, balloons • presentation ƒ stridor if lodged in trachea ƒ unilateral “asthma” if bronchial, therefore ofen misdiagnosed as asthma ƒ if totally occludes airway: cough, lobar pneumonia, atelectasis, mediastinal shif, pneumothorax, death Batteries MUST be ruled out as a foreign body (vs. coins) as they are lethal and Diagnosis and Treatment can erode through the esophagus. Batteries have a halo sign around the • any patient with suspected foreign body should be kept NPO immediately rim on AP xray and a step deformity on • inspiration-expiration chest x-ray (if patient is stable) lateral xray. • bronchoscopy or esophagoscopy with removal • rapid onset, not necessarily febrile or elevated WBC Deep Neck Space Infection

• most commonly arise from an infection of the mandibular teeth, tonsils, parotid gland, deep cervical lymph nodes, middle ear, or the sinuses Trismus means the pterygoids are involved and airway will become • ofen a rapid onset and may progress to fatal complications increasingly hard to access. Etiology • usually mixed aerobes and anaerobes that represent the fora of the oral cavity, upper respiratory tract, and certain parts of the ears and eyes These investigations should be obtained Clinical Features carefully and the surgeon should • sore throat or pain and trismus consider accompanying the patient as • dysphagia and odynophagia the worst place to lose an airway is • stridor and dyspnea during imaging. • late fndings may include dysphonia and hoarseness • swelling of the face and neck, erythema • asymmetry of the oropharynx with purulent oral discharge • lymphadenopathy Ludwig’s angina is the prototypical infection of the submandibular and Diagnosis sublingual space. • lateral cervical view plain radiograph • CT • MRI Treatment • secure the airway • surgical drainage • maximum doses of IV systemic antimicrobials regimens according to the site of infection OT47 Otolaryngology Common Medications Toronto Notes 2014

Common Medications

Table 20. Antibiotics Generic Name (Brand Name) Dose Indications Notes amoxicillin Adult: 500 mg PO tid Streptococcus, Pneumococcus, May cause rash in patients with infectious (Amoxil®, Amoxi ®, Amox®) Children: 80-90 mg/kg/d in H. influenzae, Proteus coverage mononucleosis 2 divided doses piperacillin with tazobactam 3 g PO q6h Gram-positive and negative aerobes and anaerobes May cause pseudomembranous colitis (Zosyn®) plus Pseudomonas coverage ciprofloxacin 500 mg PO bid Pseudomonas, Streptococci, MRSA, and most Do not give systemic quinolones to children (Cipro®, Ciloxan®) Gram-negative; no anaerobic coverage erythromycin (Erythrocin®, 500 mg PO qid Alternative to penicillin Ototoxic EryPed®, Staticin®, T-Stat®, Erybid®, Novorythro Encap®)

Table 21. Otic Drops Generic Name (Brand Name) Dose Indications Notes ciprofloxacin (Ciprodex®) 4 gtt in affected ear bid For otitis externa and complications of otitis media Pseudomonas, Streptococci, MRSA, and most Gram-negative; no anaerobic coverage neomycin, polymyxin B sulfate, and 5 gtt in affected ear tid For otitis externa May cause hearing loss if placed in inner ear hydrocortisone (Cortisporin Otic®) Used for inflammatory conditions which are currently infected or at risk of bacterial infections hydrocortisone and acetic acid 5-10 gtt in affected ear tid For otitis media Bactericidal by lowering pH (VoSol HC®) tobramycin and dexamethasone 5-10 gtt in affected ear bid For chronic suppurative otitis media Risk of vestibular or cochlear toxicity (TobraDex®)

Table 22. Nasal Sprays Generic Name (Brand Name) Indications Notes Steroid flunisolide (Rhinalar®) Allergic rhinitis Requires up to 4 wk of consistent use to have effect budesonide (Rhinocort®) Chronic sinusitis Long term use triamcinolonoe (Nasacort®) Dries nasal mucosa; get minor bleeding beclomethasone (Beconase®) Patient should stop if epistaxis mometasone furoate, monohydrate May sting (Nasonex®) Flonase® and Nasonex® not absorbed systemically fluticasone furoate (Avamys®) Antihistamine levocarbastine (Livostin®) Allergic rhinitis Immediate effect If no effect by 3 d then discontinue Use during allergy season Decongestant xylometazoline (Otrivin®) Acute sinusitis Careful if patient has hypertension oxymetazoline (Dristan®) Rhinitis Short term use (<5 d) phenylephrine (Neosynephrine®) If long terrn use, can cause decongestant addiction (i.e. rhinitis medicamentosa) Antibiotic/Decongestant framycetin, gramicidin, Acute sinusitis phenylephrine (Soframycin®) Anticholinergic ipratropium bromide (Atrovent®) Vasomotor rhinitis Careful not to spray into eyes as can cause burning or precipitation of narrow angle glaucoma Increased rate of epistaxis when combined with topical nasal steroids Lubricants saline, NeilMed®, Rhinaris®, Dry nasal mucosa Use prn Secaris®, Polysporin®, Vaseline® Rhinaris® and Secaris® may cause stinging Source: Dr. M. M. Carr OT48 Otolaryngology References Toronto Notes 2014

References

Textbooks Bailey BJ. Head and neck surgery-otolaryngology, 2nd ed. Philadelphia: Lippincott Williams and Wilkins, 1998. Becker W, Naumann HH, Pfaltz CR. Ear, nose, and throat diseases, 2nd ed. New York: Thieme Medical Publishers, 1994. Dhillon RS, East CA. Ear, nose, and throat, and head and neck surgery: an illustrated colour text, 2nd ed. New York: Churchill & Livingston, 1999. Jafek BW, Murrow BW. ENT secrets, 2nd ed. Philadelphia: Hanley & Belfus, 2001. Lee KJ (ed). Essential otolaryngology: head and neck surgery, 8th ed. New York: McGraw-Hill, 2003. Lucente FE, Har-El G (editors). Essentials of otolaryngology, 4th ed. Philadelphia: Lippincott Williams and Wilkins, 1999. Layland MK (ed). Washington manual otolaryngology survival guide. Philadelphia: Lippincott Williams and Wilkins, 2003. Pasha R. Otolaryngology head and neck surgery clinical reference guide, 3rd ed. San Diego: Plural Publishing, 2010.

Journal Articles American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium. Committee on hearing and equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngol Head and Neck Surg 1995;113:181-185. Ang K, Harris J, Wheeler R, et al. Human papillomavirus and survival of patients with oropharyngeal cancer. NEJM 2010;363:24-35. Berman S. Current concepts: otitis media in children. NEJM 1995;332:1560-1565. Bonner JA, Harari PM, Giralt J, et al. Radiotherapy plus cetuximab for squamous-cell carcinoma of the head and neck. NEJM 2006;354:567-568. Coker TR, Chan LS, Newberry SJ, et al. Diagnosis, microbial epidemiology, and antibiotic treatment of acute otitis media in children: a systemic review. JAMA 2010;304:2161-169. Cooper DS, Doherty GM, Haugen BR, et al. Revised American Thyroid Association Management guidelines for patients with thyroid nodules and differentiated thyroid cancer. Thyroid 2009;19:1167-1214. Desrosiers M, Evans GA, Keith PK, et al. Canadian clinical practice guidelines for acute and chronic rhinosinusitis. Allergy Asthma Clin Immunol 2011;7:2. D’Souza G, Kreimer AR, Viscidi R, et al. Case-control study of human papillomavirus and oropharyngeal cancer. NEJM 2007;356:1944-1956. Fakhry C, Westra WH, Li S, et al. Improved survival of patients with human papillomavirus-positive head and neck squamous cell carcinoma in a prospective clinical trial. J Natl Cancer Inst 2008;100:261-269. Forastiere A, Koch W, Trotti A, Sidransky D. Head and neck cancer. NEJM 2001;345:1890-1900. Furman JM, Cass SP. Benign paroxysmal positional vertigo. NEJM 1999;341:1590-6. Glasziou PP, Del Mar CB, Sanders SL, et al. Antibiotics for acute otitis media in children. Cochrane DB Syst Rev 2004;1:CDOOO219. Grégoire V, Maignon P. Intensity modulated radiation therapy in head and neck squamous cell carinoma: state of the art and future challenges. Cancer Radiother 2005;9:42-50. Hilton M, Pinder D. The Epley (canalith repositioning) maneuver for benign paroxysmal positional vertigo. Cochrane ear, nose, and throat disorders group. Cochrane DB Syst Rev 2004;Issue 4. Jackson CG, von Doersten PG. The facial nerve: current trends in diagnosis, treatment, and rehabilitation. Otolaryngol for Internist 1999;83:179-195. Li X, Gao L, Li H, Gao J, et al. Human papillomavirus infection and laryngeal cancer risk: a systemic review and meta-analysis. J Infect Dis 2013;207:479-488. Lieberthal AS, Carroll AE, Chonmaitree T, et al. The diagnosis and treatment of acute otitis media. Pediatrics 2013;e964-e999. Low DE, Desrosers M, McSherry J, et al. A practical guide for the diagnosis and treatment of acute sinusitis. CMAJ 1997;156:S1-14. MacCallum PL, Parnes LS, Sharpe MD, Harris C. Comparison of open, percutaneous and translaryngeal tracheostomies. Otolaryngol Head Neck Surg 2000;122:686-690. McIsaac WJ, Coyte PC, Croxford R, et al. Otolaryngologists’ perceptions of the indications for typanostomy tube insertion in children. CMAJ 2000;162:1285-1288. Srafford ND, Wilde A. Parotid cancer. Surg Oncol 1997;6:209-213.