Candice Colby, MD Michigan Ear Institute Central Michigan University It takes a village: The benefit of audiologic collaboration in challenging NEURotology cases Candice Colby, MD Michigan Ear Institute Central Michigan University

OUTLINE Cochlear Implantation Pediatric Unilateral Hearing Loss Emerging Candidacy Criteria Soft Failure Special Considerations in the Pediatric Population & Progressive SNHL Pediatric Unilateral Hearing Loss Special Considerations in Adult Conductive Hearing Loss

Cochlear Implantation: Emerging Candidacy Criteria

Cochlear Implants: Current Criteria Individuals 18 years of age or older Moderate to profound sensorineural hearing loss in both ears Limited benefit from amplification defined by preoperative test scores of ≤ 50% sentence recognition in the ear to be implanted and ≤60% in the opposite ear or binaurally

Cochlear Implants: EXpanding Criteria Congenitally deaf adults Multiply handicapped More residual hearing (both ears) Hybrid (mild-to-moderate hearingloss<1000Hz; severe-to-profound above >2000 Hz) Geriatric population & younger children (under the age of 1) Bilateral-Simultaneous/Sequential Unilateral hearing loss Auditory neuropathy

Finding Candidates Do you send this patient for CI eval?

Finding Candidates

Finding Candidates Testing depends on location “Best-aided condition”- does this mean with ‘hearing aids’ only (a CI is an implant and not an aid) or with addition of CI too? Test with only hearing aid? Test with CI? Test in noise?

When to Refer a Patient for a Cochlear Implant Evaluation Based upon a practice size of 2,000 patients for a PCP, it is estimated that there are 8 adult patients (4/10 of one percent prevalence) in the practice who could benefit Individuals may not learn of their candidacy from their hearing care professional A patient may be a CI candidate, if when using hearing aids, they still struggle to understand speech Referral considerations: Has difficulty understanding you or your staff on the telephone Family members routinely make telephone calls for the person When you are not facing your patient, (s)he is unable to understand you or doesn’t hear you Asks that you face them while speaking or positions themself to see your face Noise in the room (i.e., outside traffic, running water, rustling paper) affects speech understanding Family members note that your patient has difficulty hearing at home, at work, or in other situations.

Cochlear Implant: Pre-CI Assessment & Medical Evaluation Assess prior history of ear disease – particularly ear infections and history of eustachian tube dysfunction Surgical approach through facial recess requires normal middle ear space and lack of any infection Problems: perforation, cholesteatoma; infection atelectasis of tympanic membrane with contracted middle ear space Solutions: choose other ear for CI? repair TM prior to CI canal wall down approach with EAC closure

Cochlear Implantation: Soft Failure What define this?? Who decides?? Cochlear implantation failures can be grouped into the 3 broad categories of hardware, medical, and soft failures (4,5). Hardware failure involves malfunction of a cochlear implant component as ascertained via in vivo or in vitro electronic system testing. Medical failure occurs when safe operation of a cochlear implant is not possible secondary to biological factors such as flap failure, infection, or device extrusion. Soft failures, however, have not been defined by a specific cause.

Cochlear Implantation: Soft Failure prepared by a panel of experts representing the fields of otolaryngology, audiology, speech and language pathology, communica- tion science, and engineering. Representatives to the con- ference were appointed by professional organizations including the American Academy of Otolaryngology— Head and Neck Surgery, the American Otological Soci- ety, the American Neurotology Society, the William House Cochlear Implant Group, the British Cochlear Implant Group, and the European Academy of Otology and Neurotology.

Cochlear Implantation: Soft Failure All medical and programming issues should be ruled out and external components of the device exchanged with components known to be functioning properly Requires normal device imaging and integrity testing Children may have difficulty reporting aversive symptoms, and have variability in rates of hearing and language development

Cochlear Implantation: Special Considerations FOR Pediatrics & Progressive SNHL

Cochlear Implantation: Pediatrics PEDIATRIC Criteria <2 years: bilateral profound SNHL (PTA for 500, 1000 and 2000 Hz >90 dBHL >2 years: severe to profound SNHL (PTA for 500, 1000 and 2000 Hz >75 dBHL) Must verify insufficient benefit from appropriate binaural hearing aids (time? ~6 mo) Preoperative speech and language evaluation

Cochlear Implantation: Considerations Inner Ear Malformations EVAS- Enlarged Vestibular Aqueduct Syndrome Vestibular aqueduct is the bony canal containing the endolymphatic sac (ES) In contact with the dura and CSF ES contains the same fluid as the inner ear, performs absorptive and secretory functions to maintain homeostasis in the inner ear May have immunodefensive properties ‘Enlarged’ = diameter >1.5 mm (compare to posterior SCC)

Cochlear Implantation: Considerations Inner Ear Malformations 5 -15% of children with SNHL have EVA Most frequently identified anatomical Age at onset of hearing loss may range from birth to adolescence Typically fluctuates or progresses to a profound degree by early childhood Many patients will be referred for consideration for cochlear implantation 24% of EVAs are unilateral

Cochlear Implantation: Considerations Inner Ear Malformations EVAS- Enlarged Vestibular Aqueduct Syndrome Associated with Pendred Syndrome: congenital sensorineural hearing loss and euthyroid goiter (or mild hypothyroidism) Head protection required ?Avoid contact sports, scuba diving or hyperbaric oxygen treatment?

Cochlear Implantation: Pediatric Progressive SNHL Must follow these children closely: Routine, serial audiograms Speech evaluation and documentation- relative to the child AND their peers If asymmetric hearing- implant the worse hearing ear ASAP (if poor ear- implant as soon as family is comfortable) Less duration of deafness Better plasticity when younger Less social stigma If not a “traditional candidate” based on testing- may always make a case to the insurance

Pediatric Unilateral Hearing Loss

Pediatric Unilateral Hearing Loss When to amplify children? Does this change depending on the type of hearing loss? Should we operate on these children? Is there a right ear advantage?

Pediatric Unilateral Hearing Loss Compared to the SNHL group, children with unilateral aural atresia were significantly less likely to repeat a grade in elementary school (0% vs. 18.2%) yet used academic and/or amplification resources to a similar degree Conclusions: Unilateral conductive hearing loss due to aural atresia has an impact on academic performance in children, although not as profound when compared to children with unilateral SNHL Majority of these children with unilateral atresia utilize resources in the school setting Parents, educators, and health care professionals should be aware of the impact of unilateral conductive hearing loss and offer appropriate habilitative services The cosmetic deformity (microtia) is a constant reminder of the associated functional disability, so parents and teachers are motivated to pursue resources and to use them consistently to help children succeed in the classroom setting. Unilateral SNHL is a “silent” disability, so parents, teachers, and children may not be as compulsive about resource use. Additional notes: selection bias of this study. The families of children with atresia surveyed in the study had the means either to travel to Virginia for evaluation or to New York for the atresia conference selected group of predominantly Caucasian, probably middle class, children with unilateral aural atresia, grade retention rates are better than those of children with unilateral SNHL National failure rate trends have been flat, at around 9% for children in elementary education for the last 15 years (Fig. 1).18

Pediatric Conductive Hearing Loss Meta-analysis of the literature to evaluate surgical results to assist counseling patients and families

Adult Conductive Hearing Loss

Adult CHL: Evaluation With a normal ear exam, what distinguishes these entities? Acoustic reflexes Should be performed on all conductive hearing losses

Adult CHL: Semicircular Canal Dehiscence Syndrome

Adult CHL: Semicircular Canal Dehiscence Syndrome Common symptoms Audiologic: Hearing loss— most commonly low-frequency conductive or mixed Pulsatile tinnitus, autophony, aural fullness Vestibular: chronic unsteadiness, dizziness with loud noises or pressure changes Prevalence 2% of the population has thinning of the bone that can lead to SCDS Symptomatic SCD is about 0.1% of the population More commonly diagnosed in women, left side Thickness of the SCC decreases with advancing age

Adult CHL: Semicircular Canal Dehiscence Syndrome CT temporal bone with cuts perpendicular (Poschl) and parallel (Stenver) through the SCC cVEMP, oVEMP testing

Adult CHL: Semicircular Canal Dehiscence Syndrome Observation + counseling Avoid loud noise, valsalva (weight lifting, straining), pressure fluctuations in the middle ear- PE tube may help Surgical repair- reserved for patients with severely debilitating symptoms Round window plugging- closure of one of the three windows Superior canal resurfacing or plugging (middle cranial fossa or transmastoid approach) Surgical treatment for SSCD effective for vestibular symptoms, less evidence for improvement of hearing loss

Adult CHL: Semicircular Canal Dehiscence Syndrome Surgical repair