Medical Update for Audiologists

H. Alexander Arts, MD

Professor of Otolaryngology

Adult & Pediatric Otology & Neurotology

Medical Director, Cochlear Implant Program

Email: aarts@umich.edu

Conflict of Interest Disclosure

• I have no current conflicts of interest, financial or otherwise.

• I have in the past served as a paid consultant for Med-El Medical Electronics

• I am the Principal Investigator on two clinical trials sponsored by Cochlear Corp. and receive some salary support for my effort on these studies.

Overview

• Medical diagnoses relevant to audiologists– Disorders causing conductive hearing loss

• Chronic otitis media & Cholesteatoma• Otosclerosis

– Disorders causing sensorineural hearing loss• Acoustic neuroma• Neurofibromatosis Type 2• Meniere’s disease• Sudden hearing loss• Congenital hearing loss• Inner ear malformations• Superior semicircular canal dehiscence syndrome

Overview

• Medical & Surgical Treatment of Hearing Loss– Cochlear implants– Implantable bone conduction devices– Stapedectomy– Surgery for superior semicircular canal dehiscence– Management of single sided deafness

Otitis Media

Time course– Acute vs. chronic

Pathology– Serous or secretory

Chronic otitis media with effusion (COME)

– Suppurative– Mucoid– Adhesive

Acute Suppurative Otitis Media

Pus Pain Fever Conductive HL

Chronic Otitis Media

Confusing nomenclature encompassing a wide range of disease processes:– Chronic middle ear effusion– Any persistent tympanic membrane perforation– Cholesteatoma– Atelectatic disease

Chronic OM with effusion (COME)

Following AOM Eustachian tube

dysfunction Spinal fluid leak Skull base or

nasopharyngeal neoplasm Rx: atbx, steroids, surgery

(PE tubes) Be concerned about

persistent or recurrent unilateral COME

Chronic Suppurative OM, Inactive

Conductive hearing loss

Recurrent otorrhea Rx: surgery

Chronic Suppurative OM, Active When inactive chronic otitis media becomes infected Often foul smelling Sometimes visibly draining – sometimes not Rx: Topical antibiotics, often surgery

Chronic OM, Atelectatic

Cholesteatoma

Doesn’t have anything to do with cholesterol Is not a tumor Cyst lined with skin Most are acquired, but some are congenital They grow and can be destructive Can cause serious, even life threatening complications

“Safe” vs. “Unsafe” ears. Ears with cholesteatoma are unsafe.

Chronic OM, w/ Cholesteatoma

“Aquired” cholesteatoma

Chronic OM w/ Cholesteatoma

Crusts lying on the tympanic membranemay be obscuring serious pathology, oftencholesteatoma

Mastoidectomy – Often utilized in Chronic Otitis Media

Necessary for:– Disease involving the

mastoid– Surgical exposure of

another area

Two types:– Intact canal wall– Canal wall down

Usually done in conjunction with a meatoplasty

Meatoplasty

• Performed with canal wall down mastoidectomy

• Allows aeration and cleaning of the open mastoid cavity

Otosclerosis

Disease of otic capsule bone

Hereditary component

Conductive loss in normal appearing ear

This hearing lossis fixable

Stapedectomy

Tumors Causing Conductive Hearing Loss

External auditory canal skin– Squamous or basal

cell Ca

Glomus tumors– Glomus jugulare– Glomus tympanicum

Unilateral tinnitus may be due to neoplasm

Congenital aural atresia

With or without microtia

Varying degrees of EAC stenosis/atresia

Sometimes middle ear anomalies

Normal inner ear

Etiologies of Sensorineural Hearing Loss

Noise Presbycusis Ototoxicity Meniere’s disease Infection Autoimmune

Congenital Hereditary Developmental Tumors Central nervous system pathology

Noise Induced Hearing Loss

Acute vs. chronic– Acoustic trauma

Typically worse at 4kHz

Almost always symmetric

Don’t attribute unilateralhearing losses to chronic noise exposure.

Meniere’s disease

Classic symptoms:– Fluctuating/progressive hearing loss– Tinnitus– Episodic vertigo

Pathology: endolymphatic hydrops ? Endolymphatic hypertension Rx:

– Na+ restriction, diuretics– Vestibular ablation

• Surgical• Intratympanic gentamicin

Sudden SensorineuralHearing Loss (SSNHL)

A syndrome with a host of causes A viral neuritis or cochleitis in most cases

1% will have acoustic neuroma Steroids beneficial

Systemic vs. intratympanic

Antivirals may be beneficial Occasionally a sudden hearing loss can be conductive

1. We treat sudden sensorineural hearing loss as an emergency.2. Do not assume that a sudden loss is due to otitis media with effusion.3. All SSNHL patients should have an MRI.

This is important.

Congenital sensorineural hearing loss

Acquired – intrauterine insult:– Maternal rubella, CMV, syphilis, herpes, toxoplasmosis– Hyperbilirubinemia– Birth injury– Teratogenic drugs

Thalidomide, quinine, Dilantin, isoretinoin, others

Congenital sensorineural hearing loss

Hereditary about 1:4000 live births 90% autosomal recessive

– Non-syndromic– Syndromic

Dominant: Waardenburg’s, Alport’s, Pierre Robin, Crouzon’s, Treacher-Collins, BOR

Recessive: Mucopolysaccharidoses, Jervell-Lange-Nielsen, Pendred’s, Usher’s

Other: Nager, CHARGE, Goldenhar’s

Inner Ear Malformations

• Many varieties:– Enlarged vestibular aqueduct

syndrome– Mondini malformation– CHARGE syndrome

• Can present with:– Fluctuating losses– Progressive losses– Mixed losses

• Significant potential for delays due to late implantation

ENLARGED

NORMAL

Enlarged Vestibular Aqueduct Syndrome (EVAS)

Risk Factors for Newborn Hearing Loss

Family hx of hearing loss Maternal infection during pregnancy Low birth weight Hyperbilirubinemia Ototoxic medications Mechanical ventilation > 5 days APGAR scores of <5 at 1min or <7 at 5min

Neoplasms Causing SNHL

Vestibular schwannoma (acoustic neuroma)– Benign tumors of schwann cells– Rarely facial or cochlear nerve tumors

Neurofibromatosis Type II– Genetic disorder characterized by multiple schwannomas and

meningiomas– Hallmark is bilateral VS

Vestibular Schwannomaaka “Acoustic Neuroma”

Asymmetric SNHL Unilateral tinnitus Unexplained dizziness Asymmetric speech

discrimination Sudden SNHL Abnormal acoustic

reflex Abnormal ABR

Neurofibromatosis Type 2

• Often not detected until adulthood

• Have a high index of suspicion with bilateral asymmetric losses, even in children

Other Neoplasms Causing HL

Meningioma Glomus tumors (paraganglioma) Epidermoids Endolymphatic sac tumors (Von Hippel Lindau disease)

ABR vs. MRI for Diagnosis of Retrocochlear Lesions

• ABR is a sensitive screening tool for vestibular schwannoma > 1cm

• For lesions < 1cm, ABR can have up to a 15% false negative rate

• MRI is essentially 100% sensitive

• I use ABR for screening when missing a < 1cm lesion would not alter our management– Elderly– Multiple other medical morbidities

Superior Semicircular Canal Dehiscence

Superior Semicircular Canal Dehiscence

• Symptoms:– Aural fullness– Hearing loss– Pulsatile tinnitus– Autophony– Sound or pressure induced vertigo– Hyperacousis

Songer JE, Rosowski JJ. Hear Res. 2005Dec;210:53-62.

Superior Semicircular Canal Dehiscence

• Findings– Conductive or mixed hearing loss (mostly low frequency)– Supra-normal bone conduction thresholds– Tullio phenomeon

• Sound induced nystagums

– Electrocochleography• Elevated SP/AP ratio

– VEMP• Reduced thresholds

Normal tympanograms, Normal acoustic reflexes

Superior Semicircular Canal Dehiscence

Sound-Induced Eye Movement(Tullio’s Phenomenon)

500 Hz 110dBHL

Superior Semicircular Canal Dehiscence

• Etiology is unknown• Found in 0.7% of temporal bones• Sometimes induced by strain or trauma• Surgical plugging provides excellent control of vertigo

– Often improves hearing

What’s New in Cochlear Implants

• Expanded indications• Electro-acoustic stimulation (Hybrid devices)• MRI compatibility• Use in single sided deafness

Cochlear Implant Indications - Adult

• ≤ 60% Sentence recognition in best aided ear

• ≤ 50% in the ear to be implanted

• ≤ 40% for Medicare• HINT sentences at 60dB• HINT is being replaced by

AzBio• Testing in noise• Off-label implantation PTA ≥ 90dB

Cochlear Implant Indications - Children

• 12-23 mos: PTA ≥ 90dB• > 23 mos: PTA ≥ 70dB• Failure of improvement of speech and language skills

after proper amplification and aural rehab over 3 months• In older children:

– MLNT or LNT ≤ 30%

• Can also test in noise in borderline candidates

Nucleus CI422 Study

• CNC word scores– 10% - 40% in ear to be

implanted– ≤ 50% in better ear

Nucleus Hybrid Implant

• Short electrode designed for hearing preservation

• Implanted ear (poorer)– CNC words: 10% - 60%

– PTA2,3,4kHz ≥ 75dB

• Unimplanted ear (better)– CNC words:

• Better than poorer ear• ≤ 80%

• About 30% lose residual hearing

Cochlear Implants for Single Sided Deafness

Cochlear Implants forSingle Sided Deafness

• Data from bilateral CI patients leaves no doubt that some patients perform substantially better with both CI’s vs. either one individually– Studies have shown benefits from:

• Binaural summation• Binaural squelch• Localization• Alleviation of head shadow effect

• CI experience in patients with one normal hearing ear began with unilateral deafness complicated by severe tinnitus

Cochlear Implants for SSD

• Vermeire (2009)– 20 adult patients with unilateral severe tinnitus and SNHL– Demonstrable speech-in-noise benefit

• Hansen (2013)– 29 adult patients

• Included 10 with CI in combination with labyrinthectomy for Meniere’s disease

– Localization improved in most

• Gartrell (2014)– Single patient carefully studied, clearly showed improved

localization

Cochlear Implants for SSD

• Arndt (2010)– 11 adult patients with SSD– CI does not interfere with speech perception in the normal ear– Significant improvement in localization– Improved speech discrimination in noise in most conditions

Cochlear Implants in Children with SSD

• Hassepass (2012)– 3 children, 4-10 yo, non-traumatic SSD– Demonstrated improvement in localization and speech-in-noise– All children use device all waking hours at 1 year

• Plontke (2013)– Single case report: 8 yo boy after temporal bone fracture– Speech-in-noise tests improved to almost normal values in all

presentation modes– Improvement in localization was demonstrated

Cochlear Implant Illustrative Cases

• Extremely satisfied users– Complex bilateral otosclerosis patient after complete SNHL

following stapedectomy revision– Rapidly progressive bilateral Autoimmune Inner Ear Disease

(Cogan’s syndrome)

• Extremely unsatisfied user– Adult with congenital unilateral deafness

Cochlear Implants for SSD

• Bottom Line:– CI is a reasonable option for selected patients with SSD– Important considerations include:

• Recency of deafness• Etiology of deafness• Surgical status of ear• Hearing status of contralateral ear• Realistic expectations• Presence of tinnitus

Cochlear Implants and MRI

• The implanted magnet creates problems for patients needing MRI

• Until recently the magnets would have to be temporarily removed for MRI, and then replaced afterwards

• Med-El now has a clever magnet design that can be left in place for MRI’s up to 3T

Tomorrow….

aarts@umich.edu