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Worksheet Summary for Inner Ear/Sensorineural Hearing Loss

(1) Based on data and experience, develop list of otologic conditions to be targeted in the next 5 to 10 years. Include rationale.
Disease/Disorder/Condition/ Target PH Significance e.g., high prevalence, severe dz, limited treatment options Treatment Options e.g., none, ineffective, low benefit/risk ratio Disease Severity e.g., dire consequences, dire disabilities, compromised QOL Evidence Base e.g., none, some, limited Stage of readiness for research involving humans
Progressive SNHL, including age-related hearing loss Ubiquitous prevalence
- third most common chronic disorder in those over 65 and first among older males. Hearing loss is a primary determinant of function in older adults; impact is comparable to other chronic conditions affecting older population.
Hearing aids are the third most commonly used assistive device (after canes and glasses) but satisfaction is generally low. HA only partially effective and unaccepted; need for effective counseling & auditory training. Severe communication handicap with more severe hearing loss. Poorer communication abilities result in decreased QOL, increased isolation and depression for older adults with hearing loss. Variable.Evidence for limited effectiveness of HAs. Efficacy established but less compelling evidence benefit of compression amplification (NIDCD HA clinical trial). Follow-up study showed significant long-term benefit and satisfaction. Limited systematic evidence base for signal processing strategies. Limited evidence for real-world benefit of bilateral HAs. For counseling-based group rehabilitation, only short-term effectiveness established. For individual auditory training, long-term efficacy not established, effects beyond training parameters not established, and cost-effectiveness may be questionable. High level of readiness for new technology and approaches to amplification, including directional microphones for hearing aids; combining hearing aids and cochlear implants.
Individualized auditory training to maximize hearing- aid and cochlear implant benefit could be included in designs of RCTs. Genome-based association studies for age-related hearing loss.
NIHL High prevalence Unsure of preventative Rx, otoprotectives. Identification of risk factors and prevention

Speech understanding and localization impaired,

Tinnitus often accompanies, as well as hyperacusis
Limited evidence for effective treatments beyond hearing aids High
Ototoxicity Low prevalence Unsure of preventative Rx, otoprotectives. Identification of risk factors and prevention Variable severity and impact. Preliminary understanding of mechanisms, clinical parameters of prevention, and genetic susceptibility High
Fluctuating SNHL Moderate prevalence No evidence based treatment Variable severity and impact Clinical and temporal bone studies have yet to identify basic pathogenesis Intermediate
Sudden SNHL 1/5000/year Evidence based studies in progress; moderate data base of Rx efficacy; Unsure of otoprotectants Variable severity and impact.

Evidence based studies of systemic therapies are inconclusive.

Well designed studies of intratympanic therapies in progress
Congenital SNHL 1-3/1000 births HA, Cochlear implants; ID and FU poor Developmental implications in children -profound (reading, language development, psycho-social, education) Accumulating evidence of impact of early identification and intervention High
Genetic SNHL 1-2/1000 HA, Cochlear implants; ID and FU poor Developmental implications in children-profound (reading, language development, psycho-social, education) Growing databases of specific forms of auditory impairment associated with particular genotypes High for specific genetic abnormalities in which gene identification complete
Unilateral HL 1/300 includes sensorineural and conductive Management strategies employ CROS-amplification and transcranial (bone-conducted) transmission with varying levels of benefit dictated by unknown intermediary variables; delayed identification Developmental implications in children - mild to profound, though scholastic achievement effects appear to be considerable Nonrandomized, case series clinical data only Curative approaches as above. High for prosthetic approaches.
Autoimmune Ear Disease (AIED)

Low prevalence

Serious debilitating illness that rapidly leads to deafness unless treated. Occurs in children and young adults so it impacts on their future functional capacity significantly. May overlap with some cases of Meniere's disease so impact on society may be even greater.

Steroid responsiveness documented; however long term management with other anti-inflammatory medications not conclusive

Systemic steroids, immunosuppressive drugs, intratympanic glucocorticoids and other immunomodulating drugs. Most patients will require HA as a result of damage from inflammation within the inner ear. In unrelenting cases cochlear implants offer hearing rehabilitation. Surgery is not considered an option.

Progressive hearing loss to profound bilateral deafness can occur

Severe communication handicap and the potential for bilateral vestibular dysfunction
Accumulating evidence for effectiveness of high dose steroid therapy High level of readiness for new approaches in diagnosis and treatment with newer biologicals and intratympanic therapy
Tinnitus High prevalence Many treatment options, but most are ineffective

Affects general health with QOL issues

Often emotional, hearing, sleep and concentration problems.
Limited. Several RCTs conducted on pharmacotherapies, including anti-depressants, and clinical studies of electrical and magnetic stimulation and behavioral and cognitive therapies. Limited widespread benefit due to poor understanding of site(s) of injury and mechanisms. Basic research needed for understanding of mechanisms. Clinical research on humans is ongoing, including RCTs.
Vestibular Schwannoma Incidence: 1/80,000/year Surgical removal, radiation, question optimal management strategy Severe morbidity and life threatening with advanced tumor size Large but restricted to non-randomized clinical series Basic and clinical
Neurofibromatosis Type 2

Incidence: 1/500,000 between 20 and 40 years of age.

No treatment

Ineffective treatment despite intensive surgical or radiotherapy intervention. Theoretically, Erb2 adjuvant chemotherapy holds promise.

question optimal management strategy
Severe morbidity, profound deafness and loss of vestibular function along with other cranial somatic nerve paralysis. Life threatening Absence of non-randomized clinical series Basic and clinical


(2) For each target otologic condition, categorize the current state of knowledge regarding the condition and its interventions (if any) and the stage of research necessary for full development of the intervention.
Disease/ Disorder/ Condition Target Understanding of Dz/Disorder/ Condition e.g., natural hx, treatment altering natural course Understanding of patho-physiology Is there a therapeutic target Describe intervention: any modality e.g., surgery, drug, device, drug, behavior
Progressive SNHL; NIHL; Ototoxicity; Sudden SNHL; Fluctuating SNHL; Congenital & Early Onset SNHL, Genetic, Unilateral, AIED

Epidemiology studies on all SNHL. Define subtypes (etiology, genomic, phenotype, mechanism) of SNHL and their natural history to reduce heterogeneity of SNHL

AIED: Epidemiology studies on one cohort of 116 patients

Potential for genetic predisposition, some evidence of HLA factors

While some contribution of genetics is understood, role of genetics to most SNHL is poorly understood. More importantly, we need to understand better the interaction with genetic predisposition and environmental interactions.

AIED: There are clear data that suggest that some patients have an autoimmune background with other rheumatic disorders coexisting. The mechanism underlying these patients compared to those in which the ear is the primary target needs to be better understood

Some data implicated B cell humoral immunity is at play and other data implicates T cells - clarification of this is imperative since targeting therapy would differ.

Inner Ear, Peripheral and Central Auditory Pathways, Behavioral

AIED: Inner Ear

(68 kD) antigen that is present in both cochlea and vestibular labyrinth. Some data suggest that it is a HSP-70 antigen as well as other data indicating that it is a choline-like transport protein

Role of otoprotective agents needs to be studied. Role of molecular and biological therapy (gene Rx, inner ear regeneration, neural regeneration, stem cells, etc.) needs to be investigated.
Drug, Molecular and Gene product delivery systems
Behavioral Interventions (Counseling-based and individual auditory training)
Devices interventions (hearing aids, cochlear implants, multimodal)
National Registry and Networking of Newborn Screening Programs

AIED: Clinical trial utilizing MTX was found not to be effective. Newer biological modifying drugs are promising but unproven in this disorder. Some recent work with intratympanic corticoids may prevent systemic side effects of the only proven drug in this disorder, prednisone or other glucocorticoids
Tinnitus Limited understanding of natural history Limited information on emotional and organic components of heterogeneous disorder

Peripheral and Central, Behavioral

Pharmacotherapy; Behavioral Therapy; Device-based Therapy
Vestibular Schwannoma Growing evidence of understanding regulatory genes responsible. Case series data available on natural history, outcomes with surgery and radiation. Likely due to faulty expression of MERLIN protein that normally inhibits cell proliferation Genetic abnormality; tumor Need to define optimal intervention strategy: observation, radiotherapy, surgical intervention
Develop prospective longitudinal trial to evaluate treatment
Need National Registry
Neurofibromatosis Type 2 Growing evidence of understanding regulatory genes responsible. Case series data available on natural history, outcomes with surgery and radiation. Need for early identification Somatic mutations of the NF2 tumor suppressor gene (on Chromosome 22) responsible for the pathogenes is of both familial and non-familial vestibular schwannoma; mechanism of tumorigenes is complex with a 'two-hit' mutation model affecting MERLIN protein. 2003 NINDS Workshop: Developing Therapies for the Neurofibromatoses. Genetic abnormality; tumort Need to define optimal intervention strategy: observation, radiotherapy, surgical intervention


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