Ear Structures of importance Otologic Dizziness (Dizziness from Ear)

Timothy C. Hain, MD

Northwestern University, Chicago [email protected]

The ear is an inertial navigation Vestibular Reflexes device n Semicircular Canals n VOR: Vestibulo- are rate sensors. ocular reflex n Otoliths (utricle and n VSR: Vestibulospinal saccule) are linear reflex accelerometers n Bilateral symmetry means redundant design.

Positional Vertigo Otologic (Ear) Dizziness The most common syndrome n BPPV (benign paroxysmal n positional vertigo) -- about Benign Paroxysmal 50% of otologic, 20% all n Meniere’s disease -- about 20% Positional Vertigo n Vestibular neuritis and related conditions (15%) (BPPV) -- bed spins n Bilateral vestibular loss n Orthostatic hypotension (dizzy upright) (about 1%) n n SCD and Fistula (rare but Central positional nystagmus (dizzy everywhere) worth knowing) n Low CSF pressure syndrome (dizzy upright)

1 Benign Paroxysmal Positional Positional Vertigo Vertigo (BPPV) Dix-Hallpike Maneuver

61 Y/O man slipped on wet floor. LOC for 20 minutes. In ER, unable to sit up because of dizziness Hallpike Maneuver: Positive

Benign Paroxysmal Positional BPPV Mechanism: Utricular debris migrates to Vertigo (BPPV) posterior canal n 20% of all vertigo n Brief and strong n Provoked by change of head position n Definitively diagnosed by Hallpike test

BPPV treatment Unilateral Vestibular n Medication (e.g. antivert) – n Vestibular Neuritis/Labyrinthitis (common) minor benefit n Meniere’s disease (unusual, 1/2000 – May avoid vomiting by pretreating prevalence) n n Excellent response to PT Acoustic Neuroma (rare) n Surgery – canal plugging if n Vestibular paroxysmia (not sure how rehab fails (need more common) rehab after plug). Rarely done.

2 Vestibular Spontaneous Nystagmus Vestibular Neuritis: Case seen with video Frenzel Goggles

56 y/o woman began to become dizzy after lunch. Dizziness increased over hours, and consisted of a spinning “merri-go-round” sensation, combined with unsteadiness. Vomiting ensued 2 hours later, and she was brought by family members to the ER.

Vestibular Spontaneous Nystagmus recorded on ENG Aside : how to examine for SN (Electronystagmography)

n Frenzel Goggles (best) n Ophthalmoscope (good –but backwards) n Gaze-evoked nystagmus (pretty good) n Sheet of white paper (neat)

Vestibular Neuritis -- rx Meniere’s Disease n Disturbance of unknown cause (Viral ? Vascular) n involving vestibular nerve Prosper Meniere or ganglion – Fluctuating hearing n Disability typically lasts 2 weeks. – Episodic Vertigo n Steroids if dx first 2 days – Fluctuating (roaring) Tinnitus n Symptomatic Rx – Aural Fullness (meclizine, phenergan, benzodiazepine) n About 1/2000 people in population n Rehab if still symptomatic n after 2 months. Chronic condition – lasts lifetime n These patients can still get BPPV !

3 Etiology of Meniere’s (Dogma) Meniere’s disease – symptoms n Dilation and episodic rupture of inner ear membranes (Endolymphatic Hydrops) n Progressive hearing loss -- usually go deaf n As endolymph volume and pressure increases, the n utricular/saccular and Reissner’s membranes rupture, Episodic vertigo – out of commission for releasing potassium-rich endolymph into the perilymph several days causing cochlear/vestibular paralysis n Ataxia – gradually increases over years n Visual sensitivity à

Visual Sensitivity is common Otolithic Crises of Tumarkin n Sensory integration n Drop attacks disorder – upweight n Go from upright to vision, downweight on floor in fraction everything else of second n Grocery store, n No LOC Omnimax, Target, etc n Very dangerous n Typical of disorders n with intermittent Destructive vestibular problems treatment is best

Treatments of Menieres Acoustic Neuroma n Medical management – – Usually ineffective n Bad rehab candidate while fluctuating n Surgery – Low dose gentamicin treatment works 85% – High dose gentamicin treatment (overkill) n Rehab useful post destructive treatment

Hain TC, Ostrowski T. Unsteady Influence. Menieres disease. Advances for directors in rehabilitation October 2007, 51-51

4 Acoustic Neuroma Treatment of Acoustic Neuroma

n Cause of unilateral n Watchful waiting (about 25%) vestibular loss n Operative removal (about 50%) – losing n Rare cause of ground unilateral loss n n Generally also deaf on Gamma Knife (about 25%) – gaining one side ground because effective and noninvasive n Slowly progressive – n Good rehab candidate little or no vertigo

Vestibular Paroxysmia (AKA Clinical Diagnosis of MVC microvascular compression)

n Quick spins n Irritation of vestibular nerve n May have nystagmus on hyperventilation n Quick spins, tilts, dips n Response to anticonvulsant n Motion sensitivity n No rehab potential n May follow 8th nerve surgery, Gamma knife treatment, acoustic neuroma n Wastebasket syndrome in some cases ?

Bilateral Vestibular Loss SYMPTOMS OF BILATERAL VESTIBULAR LOSS A stewardess developed a toe-nail infection. She l OSCILLOPSIA underwent course of gentamicin and vancomycin. 12 days after starting therapy she developed imbalance. 21 days after starting, she was “staggering like a drunk person”. Meclizine was prescribed. Gentamicin was stopped on day 29. One year later, the patient had persistent imbalance, visual symptoms, and had not returned to work. Hearing is normal. She unsuccessfully sued her doctor for malpractice.

5 SYMPTOMS OF BILATERAL Bilateral Vestibular Loss VESTIBULAR LOSS Causes:

n Ototoxicity ! l ATAXIA n Bilateral forms of unilateral disorders (e.g. bilateral vestib neuritis) n Congenital (e.g. Mondini malformation) n idiopathic

N=43, NMH 1990-1998

Dynamic Illegible ‘E’ test DIAGNOSIS IS EASY (DIE test) l History of recent IV antibiotic medication n l Distance vision with head Eyes closed tandem Romberg is positive still l Dynamic illegible ‘E’ test (DIE) failed n Distance vision with head moving n Normal: 0-2 lines change. n Abnormal: 4-7 lines ----> change

LABORATORY DIAGNOSIS Rapid Dolls failed Everything should be “dead” n VOR: Vestibulo- ocular reflex l ENG l Rotatory chair l VEMP

6 DIAGNOSIS Continued DIAGNOSIS Continued l Rotatory chair confirms diagnosis but requires cooperation l ENG shows little or no response

Perilymph Fistula and SCD (superior Treatment Bilateral canal dehiscence)

Fluctuating conditions n No medical management (other than avoiding more damage) No rehab until after surgery n Outstanding rehab candidate n Superior Canal n Be prepared for a deposition Dehiscence

Case: WS Tullio in SCD Retired plastic surgeon, with impaired hearing related to war injuries, found that when he went to church, when organ was playing, certain notes made him stagger. His otolaryngologist noted that during audiometry (with hearing aid in), certain tones reliably induced dizziness and a mixed vertical/torsional nystagmus. This “Tullio’s phenomenon” could be easily reproduced experimentally. MRI scan was normal.

7 Valsalva in SCD

Superior Canal Dehiscence Diagnosis of SCD n Etiology: – Congenital bone defect n (2% ?) History of sound and pressure sensitivity – Trauma may n Valsalva test is easiest bedside test exacerbate n n Treatment: Temporal Bone CT scan (high resolution) – Surgical n VEMP: Vestibular evoked myogenic potentials » Plug » Resurface

Case: KF A large round window fistula was found and symptoms completely resolved after a second surgery. •After SCUBA diving, a young woman developed vertigo, aural fullness and tinnitus for 1 year. •Symptoms were worsened by tragal pressure and straining. Surgery was performed.

8 Summary of Otologic Vertigo More details

Hain, T.C. Approach to the patient with Dizziness and Vertigo. Practical Neurology (Ed. Biller), Lippincott- Raven

More movies www.dizziness-and-hearing.com

9