ONLINE EXCLUSIVE

Kristen Grine, DO; A guide to managing disorders Mark Stephens, MD Department of Family and Community Medicine, Penn of the ear pinna and canal State College of Medicine, University Park

mstephens3@ This review will help you troubleshoot everything from pennstatehealth.psu.edu infections and foreign bodies to trauma and neoplasm. The authors reported no potential conflict of interest relevant to this article.

hich antibiotics are most useful for infection follow- PRACTICE ing ear piercing? When is it safe to attempt removal RECOMMENDATIONS of a foreign body from the ear canal, and which ceru- ❯ Prescribe topical antibiotics W menolytic agent may be best for ear wax? This review covers com- for uncomplicated otitis externa, reserving systemic mon ailments of the outer ear, which are often readily diagnosed agents for infection given a patient’s history and thorough physical examination. We extending outside the ear also address more complicated matters such as deciding when canal, necrotizing otitis to refer for treatment of suspected malignant otitis externa, and externa, or patients who which lab markers to follow when managing it yourself. are immunodeficient. C ❯ Avoid clearing cerumen if A (very) brief review of ear anatomy a patient is asymptomatic Understanding the unique embryology and intricate anatomy and advise patients/parents of the external ear informs our understanding of predictable on Do’s and Don’ts for ear infections, growths, and malformations. wax accumulation. C The external ear is composed of the external auditory ❯ Consider flooding the canal and auricle. The external auditory canal has a lateral ear canal with xylocaine, (external) cartilaginous portion and a medial (internal) bony alcohol, or mineral oil before portion. The auricular structure is complex and formed by the attempting insect removal. C helix, antihelix (crura; scaphoid fossa), tragus, antitragus, con-

Strength of recommendation (SOR) chae, and lobule. The auricle is composed of elastic cartilage A Good-quality patient-oriented covered by skin. The lobule is composed of skin, adipose tis- evidence sue, and connective tissue. B Inconsistent or limited-quality Embryologically, the auricle, auditory canal, and middle patient-oriented evidence ear form from ectoderm of the first 2 branchial arches during  C Consensus, usual practice, opinion, disease-oriented early gestation. The auricle forms from the fusion of soft-tissue evidence, case series swellings (hillocks). Three hillocks arise from the first branchial arch and 3 from the second branchial arch during the fifth and sixth weeks of gestation. Tissues from the second branchial arch comprise the lobule, antihelix, and caudal helix. The cartilage of the tragus forms from the first branchial arch. The ear canal forms from an epithelial invagination of the first branchial arch that also occurs during the fifth week of gestation.1

Infections Perichondritis Inflammation or infection of the connective tissue layer sur-

MDEDGE.COM/FAMILYMEDICINE VOL 69, NO 6 | JULY/AUGUST 2020 | THE JOURNAL OF FAMILY PRACTICE E1 rounding the auricular cartilage (perichon- due to allergic conditions, and placement of drium) results in perichondritis. Further hearing-aid devices.6 extension of infection can lead to an auricu- ❚ What you’ll see. Suspect AOE when lar abscess. Both of these conditions can have signs or symptoms of ear canal inflamma- serious consequences. tion have appeared rapidly (generally within ❚ What you’ll see. The most common 2 days) over the past 3 weeks.7 Findings in- risk factor for perichondritis is the popular clude otalgia, itching, fullness, tragal ten- practice of cosmetic transcartilaginous pierc- derness, ear canal edema, erythema with or ing.2 Piercing of the helix, scapha, or anti- without otorrhea, lymphadenitis, or cellulitis helix (often referred to as “high” ear piercing) of the pinna or adjacent skin.7 AOE must be causes localized trauma that can strip the ad- distinguished from other causes of otalgia jacent perichondrium, decrease blood sup- and otorrhea, including dermatitis and viral ply, create cartilaginous microfractures, and infection. lead to devascularization. Rates of infection ❚ How to treat. Topical therapy is rec- as high as 35% have been reported with high- ommended for the initial treatment of un- ear piercing.3 complicated AOE, usually given over 7 days. The most common microbes associated Multiple topical preparations are available, with perichondritis and pinna abscess for- such as ciprofloxacin 0.2%/hydrocortisone mation are Pseudomonas and Staphylococcus 1.0%; neomycin/polymyxin B/hydrocorti- Rates of species.2 P aeruginosa accounts for a majority sone; ofloxacin 0.3%; or acetic acid 2.0%.7 infection as high (87%) of post-piercing infections of the au- Avoid these agents, though, if you suspect as 35% have ricular cartilage.2 tympanic membrane rupture. Quinolone been reported ❚ How to treat. The cornerstone of treat- drops are the only topical antimicrobials ap- with high-ear ment is early detection and antimicrobial proved for middle ear use.7 piercing. coverage with antipseudomonal antibiotics. Systemic antibiotics are not recommend- Ciprofloxacin is the oral antibiotic of choice ed for the initial treatment of AOE. Topical because of its ability to penetrate the tis- agents deliver a much higher concentration sue.4 Other options include clindamycin and of medication than can be achieved systemi- third- or fourth-generation cephalosporins. cally. Consider systemic antibiotics if there is If the wound becomes abscessed, perform­ extension outside the ear canal, a concern for (or refer for) early surgical incision and necrotizing otitis externa (more on this in a drainage.5 A failure to promptly recognize bit), or the patient is immunodeficient.8 perichondritis or to mistakenly prescribe non-­ Patient (or parent) education is impor- antipseudomonal antibiotics contributes to tant to ensure proper medication adminis- increased rates of hospitalization.2 Cosmetic tration. The patient should lie down with the deformity is the most common complication affected ear facing up. After the canal is filled of perichondritis. This may require recon- with drops, the patient should remain in this structive surgery. position for 3 to 5 minutes. Gently massag- ing the tragus can augment delivery. Patients Otitis externa should keep the ear canal as dry as possible Acute otitis externa (AOE; “swimmer’s ear”) and avoid inserting objects (eg, hearing aids, is cellulitis of the skin and subdermis of the ear buds, cotton-tipped applicators) into the external ear canal. It is most prevalent in canal for the duration of treatment. The deliv- warm, moist climates and almost always as- ery of topical antibiotics can be enhanced by sociated with acute bacterial infection, most wick placement. Prescribe analgesics (typi- commonly P aeruginosa or S aureus.6 There is cally nonsteroidal anti-inflammatory agents) also an increased association with poor water based on severity of pain.7 quality (containing higher bacterial loads). Have patients abstain from water sports Anything breaching the integrity of the ear for 7 to 10 days. Showering is acceptable canal can potentially predispose to the de- with minimal ear exposure to water; bath- velopment of AOE. This includes trauma ing is preferred when possible. If there is no from cleaning, cerumen removal, scratching clinical improvement in 48 to 72 hours, ask

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patients to return for re-evaluation.8 Preven- (particularly if CT findings are normal and tion is essential for patients with a history of clinical suspicion is high) and for follow-up recurrent otitis externa. Acetic acid solutions with treatment. create an acidic environment within the ca- ❚ How to treat. Management involves a nal to help prevent recurrent AOE. Ear plugs team approach with otolaryngology, radiolo- and petroleum jelly–soaked cotton plugs pri- gy, neurology, endocrinology, and infectious or to water exposure may also help prevent disease specialists. Long term (6-8 weeks) recurrent AOE. antipseudomonal antibiotic treatment is typical. Malignant otitis externa Let culture results guide the choice of Malignant, or necrotizing, otitis externa is an antibiotic. Fluoroquinolone therapy, usually aggressive disease form of otitis externa that ciprofloxacin, is used most often.12 Surgical is most common in individuals with diabetes intervention may be required for local de- or other immunodeficiency disorders.9 Most bridement and drainage of abscesses. Close cases are due to infection with P aeruginosa.10 follow-up is necessary due to reports of re- Prior to the availability of effective antibiot- currence up to 1 year after treatment. If left ics, mortality rates in patients with necrotiz- untreated, necrotizing otitis externa can lead ing otitis externa were as high as 50%.11 to osteomyelitis, meningitis, septic thrombo- ❚ What you’ll see. Patients typically sis, cerebral abscess, and death.11 present with severe ear pain, otorrhea, con- In head-to- ductive hearing loss, and a feeling of fullness head laboratory in the external ear canal. Physical examina- Cerumen impaction comparisons, tion reveals purulent otorrhea and a swollen, The relatively small diameter of the external distilled water tender ear canal. Exposed bone may be vis- auditory canal increases the risk for impac- appears to ible, most often on the floor of the canal. The tion of cerumen and foreign bodies. Ceru- be the best tympanic membrane and middle ear are sel- men impaction, in particular, is a common cerumenolytic. dom involved on initial presentation. primary care complaint. Cerumen forms The infection often originates at the junc- when glandular secretions from the outer tion of the bony and cartilaginous portion of two-thirds of the ear canal mix with exfoliat- the external canal, spreading through the ed skin. It functions as a lubricant for the ear fissures of Santorini to the skull base. If not canal and as a barrier against infection, water aggressively treated, the infection spreads accumulation, and foreign bodies.13 medially to the tympanomastoid suture caus- ❚ What you’ll see. You may encoun- ing intracranial complications—usually a fa- ter cerumen impaction in an asymptomatic cial nerve neuropathy. patient when it prevents visualization of the Given these clinical findings, promptly external auditory canal or tympanic mem- order laboratory studies and imaging to con- brane, or when a patient complains of con- firm the diagnosis. The erythrocyte sedimen- ductive hearing loss, tinnitus, dizziness, ear tation rate and C-reactive protein level are pain, itching, and cough.13 It is found in 1 in typically elevated, and either can be used 10 children and 1 in 20 adults.13 There is a as a marker to follow treatment. Computed higher incidence in patients who are elderly, tomography (CT) helps to determine the lo- are cognitively impaired, or wear hearing de- cation and extent of disease and is recom- vices or ear plugs.13,14 Asymptomatic cerumen mended as the initial diagnostic imaging impaction should not be treated. A recent modality for patients with suspected malig- clinical guideline provides a useful “do and nant otitis externa.12 don’t” list for patient education (TABLE).13 Magnetic resonance imaging helps de- ❚ How to treat. In asymptomatic pa- fine soft-tissue changes, dural enhancement, tients, the presence of cerumen on examina- and involvement of medullary bone, making tion is not an indication for removal. Based this the preferred modality to monitor thera- on current guidelines,13 impacted cerumen peutic response.12 Technetium bone scan- can safely be removed from the ear canal of ning can also be used for the initial diagnosis symptomatic patients in several ways:

MDEDGE.COM/FAMILYMEDICINE VOL 69, NO 6 | JULY/AUGUST 2020 | THE JOURNAL OF FAMILY PRACTICE E3 TABLE Do’s and don’ts for patients13 Do Don’t Leave earwax alone if not causing symptoms Excessively clean your ear canal Remove ear wax if it … Place small objects in the ear, including cotton tips. - affects your hearing - causes ringing in your ear - causes irritation - causes hearing device malfunction See your health care provider if you experience Use ear candles ear pain, drainage, or bleeding Keep hearing devices clean Use drops if eardrum could be/is ruptured

• Manual removal with cerumen loop/ (typically beads, cotton tips, and insects) are spoon or alligator forceps. This meth- more common in children than adults.16 od decreases the risk for infection ❚ What you’ll see. Most foreign bodies because it limits moisture exposure. are lodged in the bony part of the external Insect removal However, it should be performed by a auditory canal, and many patients try to re- can be facilitated health care provider trained in its use move the object before seeking medical care. by first flooding because of the risk for trauma to the Removal requires adequate visualization the canal with ear canal and tympanic membrane. and skill.17 Although patients may be asymp- xylocaine, • Irrigation of the ear using tap wa- tomatic, most complain of pain, fullness, de- alcohol, or ter or a 50-50 solution of hydrogen creased hearing, or otorrhea. mineral oil. peroxide and water. Irrigation can be ❚ How to treat. Directly visible objects can achieved with a syringe or jet irrigator often be removed without referral. Suction, ir- using a modified tip. This method also rigation, forceps, probes, and fine hooks have has a risk for trauma to the ear canal been used. Insect removal can be facilitated by and tympanic membrane and should first flooding the canal with xylocaine, alcohol, only be performed by appropriately or mineral oil. Acetone may be used to dissolve trained health care professionals. foreign bodies containing Styrofoam or to loos- • Use of cerumenolytic agents to en glues. If the object is a button battery, avoid soften and thin earwax and promote irrigation to prevent liquefaction tissue necrosis. natural extrusion. Several types of ce- Complications of foreign body removal rumenolytic drops (water-based and include pain, otitis externa, otitis media, oil-based) are available and appear and trauma to the ear or tympanic mem- to be equally effective. Water-based brane. The likelihood of successful removal solutions contain hydrogen peroxide, of the object decreases and the risk for com- docusate sodium, acetic acid, and so- plications increases with each subsequent dium bicarbonate. Oil-based drops attempt.17 Consult an otolaryngologist if se- may contain peanut, almond, or olive dation or anesthesia is required, the foreign oils. A thorough allergic history should body is tightly wedged, there is trauma to the be performed to avoid using products ear canal or tympanic membrane, the foreign in patients with nut allergies. In head- body has a sharp edge (eg, glass or wire), or to-head laboratory comparisons, removal attempts have been unsuccessful. distilled water appears to be the best cerumenolytic.15 Trauma Sports injuries, motor vehicle accidents, Foreign bodies bites, falls, and burns are the primary causes Foreign bodies in the external auditory canal of trauma to the external ear.18

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❚ What you’ll see. Blunt auricular trau- diation predisposes patients to the develop- ma predisposes to infection, necrosis, and ment of squamous cell carcinoma (SCC) and scar contracture. One of the most common melanoma. SCC has a variety of presenta- sequelae is cauliflower ear. Trauma is par- tions including papules, plaques, and nod- ticularly common with contact sports such ules. SCC has a higher metastatic potential as boxing, wrestling, or mixed martial arts. than does BCC. The skin of the auricle attaches directly to the perichondrium. Following blunt or shear- ing trauma to the auricle, hematomas form Keloid within the space between the perichondrium Keloids are an abnormal healing response and cartilage of the anterior ear.19 Following to soft-tissue injury: benign fibrocartilagi- hematoma formation, the healing process nous growths that extend beyond the original brings chondrocytes and fibroblasts to the wound. subperichondrial space, promoting fibrocar- ❚ What you’ll see. Keloids are more com- tilage formation. Over time (and with repeat- mon in dark-skinned individuals and tend to ed injury), this can lead to a chronic, bulbous result from burns, surgical incisions, infec- deformity known as cauliflower ear. tion, trauma, tattooing, injections, piercings, ❚ How to treat. Small hematomas can and arthropod bites. In some cases, they arise be managed by aspiration, while larger ones spontaneously. Keloids are more common in generally require open drainage.20 Newer areas of increased skin tension (chest, shoul- If the object in treatments involving pressure dressings and ders, back), but may occur on the ears—most the external the use of fibrin glue have been proposed.20 commonly after piercing or trauma. Keloids auditory canal is Recommend that athletes participating in present clinically as slow-growing rubbery or a button battery, contact sports wear appropriate protective firm nodules. The diagnosis is typically based avoid irrigation headgear to prevent auricular hematoma and on clinical appearance but can be confirmed to prevent cauliflower ear. by histopathology. liquefaction ❚ How to treat. Treatments vary and tissue necrosis. include observation, excision, intralesional Neoplasm injections, cryotherapy, enzyme therapy, Roughly 5% of all skin cancers involve the ear, silicone gel application, and irradiation.23 most frequently the pinna due to chronic sun Recurrence is common; no therapy has exposure.21 The most frequently occurring been proven to be universally superior or malignancy of the external ear is basal cell preferred. carcinoma (BCC), which is responsible for 80% of all nonmelanoma skin cancers.22 ❚ What you’ll see. BCC of the ear usu- Congenital malformations ally involves the preauricular area and the Atresia helix. The risk for BCC is related to exposure Disruption of embryologic development to ultraviolet radiation. BCC of the ear is (failed invagination of the external auditory more common in men and can be particu- canal) can lead to a stenotic or absent ear ca- larly aggressive, highlighting the importance nal (aural atresia). Aural atresia is also often of prevention and prompt recognition. BCC associated with fusion of the incus and mal- typically presents as a fleshy papule that is of- leus. This condition occurs predominantly ten translucent or “pearly’” and has overlying in males. Unilateral atresia is more common telangiectasia and a “rolled” border. Central than bilateral atresia, and the right ear is ulceration can occur as well. more often involved than the left.24 ❚ How to treat. Usual treatment of BCC is surgical excision. Prevention is critical and Microtia centers on sun avoidance or the use of appro- Microtia is the incomplete development of priate sunscreens. the pinna leading to a small or deformed In addition to BCC, exposure of the pinna. Microtia can be unilateral or bilateral. external ear to sunlight and ultraviolet ra- As with atresia, microtia more commonly af-

MDEDGE.COM/FAMILYMEDICINE VOL 69, NO 6 | JULY/AUGUST 2020 | THE JOURNAL OF FAMILY PRACTICE E5 fects males and, if unilateral, the right side is ing future directions for microtia research. Eur J Med Genet. 2014;57:394-401. more often affected than the left. Microtia can 2. Sosin M, Weissler JM, Pulcrano M, et al. Transcartilaginous ear occur in isolation but is often associated with piercing and infectious complications: a systematic review and critical analysis of outcomes. Laryngoscope. 2015;125:1827- genetic syndromes such as Treacher Collins 1834. syndrome and craniofacial microsomia (Gold- 3. Stirn A. Body piercing: medical consequences and psychologi- cal motivations. Lancet. 2003;361:1205-1215. enhar syndrome). When microtia is identified 4. Liu ZW, Chokkalingam P. Piercing associated perichondri- (typically at birth or early infancy), audiologic tis of the pinna: are we treating it correctly? J Larygol Oncol. 2013;127:505-508. testing and a thorough physical examination 5. Mitchell S, Ditta K, Minhas S, et al. Pinna abscesses: can we for evidence of associated defects should be manage them better? A case series and review of the literature. Eur Arch Otorhinolaryngol. 2015;272:3163-3167. performed. Consult with an audiologist, clini- 6. Stone KE. Otitis externa. Pediatr Rev. 2007;28:77-78. cal geneticist, or pediatric otolaryngologist. 7. Rosenfeld RM, Schwartz SR, Cannon CR, et al. Clinical practice guideline: acute otitis externa. Otolaryngol Head Neck Surg. 2014;150(1 suppl):S1-S24. Pre-auricular pits 8. Prentice P. American Academy of Otolaryngology: Head and Neck Surgery Foundation clinical practice guideline on acute Pre-auricular pits (sinuses) are tiny indenta- otitis externa. Arch Dis Child Educ Pract Ed. 2015;100:197. tions anterior to the helix and superior to the 9. Unadkat S, Kanzara T, Watters G. Necrotising otitis externa in the immunocompetent patient. J Laryngol Otol. 2018;132:71-74. tragus. While pre-auricular pits are more com- 10. Carfrae MJ, Kesser BW. Malignant otitis externa. Otolarngol Clin mon on the right side, they are bilateral in 25% N Am. 2008;41:537-549. 25 11. Chandler JR, Malignant otitis externa. Laryngoscope. to 50% of cases. Pre-auricular pits occur in up 1968;78:1257-1294. to 1% of white children, 5% of black children, 12. Hollis S, Evans K. Management of malignant (necrotising) otitis Directly visible and 10% of Asian children.25 Children with this externa. J Laryngol Otol. 2011;125:1212-1217. 13. Schwartz SR, Magit AE, Rosenfeld RM, et al. Clinical practice foreign objects condition should undergo formal audiologic guideline (update): earwax (cerumen impaction). Otolaryngol can often testing as their risk for hearing loss is higher Head Neck Surg. 2017;156:S1-S29. 14. Guest JF, Greener MJ, Robinson AC, et al. Impacted cerumen: 26 be removed compared with the general population. composition, production, epidemiology and management. without The branchio-oto-renal syndrome (as- QJM. 2004;97:477-488. 15. Saxby C, Williams R, Hickey S. Finding the most effective ceru- referral, but sociated with pre-auricular pits and hearing menolytic. J Laryngol Otol. 2013;127:1067-1070. loss) also features structural defects of the ear, 16. Awad AH, ElTaher M. ENT foreign bodies: an experience. Int the likelihood Arch Otorhinolaryngol. 2018;22:146-151. of success renal anomalies and/or nasolacrimal duct 17. Heim SW, Maughan KL. Foreign bodies in the ear, nose, and decreases with stenosis or fistulas. If this syndrome is sus- throat. Am Fam Physician. 2007;76:1185-1189. 18. Sharma K, Goswami SC, Baruah DK. Auricular trauma and each subsequent pected, renal ultrasound imaging is warrant- its management. Indian J Otolaryngol Head Neck Surg. attempt. ed. Other indications for renal ultrasound in 2006;58:232-234. 19. Haik J, Givol O, Kornhaber R, et al. Cauliflower ear–a minimally patients with a pre-auricular pit are any dys- invasive treatment in a wrestling athlete: a case report. Int Med morphic feature, a family history of deafness, Case Rep J. 2018;11:5-7. 20. Ebrahimi A, Kazemi A, Rasouli HR, et al. Reconstructive sur- an auricular malformation, or a maternal his- gery of auricular defects: an overview. Trauma Mon. 2015;20: tory of gestational diabetes.27 Pre-auricular e28202. 21. Warner E, Weston C, Barclay-Klingle N, et al. The swollen pinna. pits do not require surgery unless they drain BMJ. 2017; 359; j5073. chronically or become recurrently infected. 22. Rubin AI, Chen EH, Ratner D. Basal cell carcinoma. N Engl J Complete surgical excision is the treatment Med. 2005;353:2262-2269. 23. Ranjan SK, Ahmed A, Harsh V, et al. Giant bilateral keloids of the of choice in these cases. JFP ear lobule: case report and brief review of the literature. J Family Med Prim Care. 2017;6:677-679. 24. Roland PS, Marple BF. Disorders of the external auditory canal. CORRESPONDENCE J Am Acad Audiol. 1997;8:367-378. Mark Stephens, MD, 1850 Park Avenue, State College, PA 25. Scheinfeld NS, Silverberg NB, Weinberg JM, et al. The preauric- 16801; [email protected] ular sinus: a review of its clinical presentation, treatment, and associations. Pediatr Dermatol. 2004;21:191-196. 26. Roth DA, Hildesheimer M, Bardestein S, et al. Preauricular skin tags and ear pits are associated with permanent hearing impair- ment in newborns. Pediatrics. 2008;122:e884-890. References 27. Tan T, Constantinides H, Mitchell TE. The preauricular sinus: a 1. Cox TC, Camci ED, Vora S, et al. The genetics of auricular develop- review of its aetiology, clinical presentation and management. ment and malformation: new findings in model systems driv- Int J Ped Otorhinolaryngol. 2005;69:1469-1474.

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