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Auditory Neuropathy Spectrum Disorder Gail Padish Clarin, Aud ANATIONAL RESOURCE CENTER GUIDE FOR FOR EARLY HEARING HEARING ASSESSMENT DETECTION & & MANAGEMENT INTERVENTION Chapter 8 Auditory Neuropathy Spectrum Disorder Gail Padish Clarin, AuD uditory Neuropathy Spectrum (8th nerve). They advocate use of the term Use of the term Disorder (ANSD) is a term “neural hearing loss” to describe pathology “neuropathy” to describe Arecently recommended by an in spiral ganglion cells or the central this disorder has resulted expert panel in the audiology profession nervous system and “sensory hearing loss” in much discussion in the to describe hearing loss characterized by to describe loss resulting from pathology normal or near normal cochlear hair cell in the hair cells. audiology profession. function and absent or abnormal auditory nerve function.1 Difficulty hearing in noise, In an effort to be consistent with recent fluctuating hearing, and speech perception terminology used in the audiology performance not predicted by level of profession regarding this type of hearing residual hearing have been reported loss, the term ANSD will be utilized (Starr et al., 1996; Rance et al., 1999). in this chapter. The vast etiologies of The incidence of ANSD in children with ANSD result in a heterogeneous group severe-profound hearing loss has been of patients—each who must be managed reported as 13.4% (Sanyebhaa et al., 2009). methodically and individually for optimal communication and developmental Use of the term progress. An understanding of what is “neuropathy” to describe known about ANSD and what remains this disorder has resulted unknown will assist the pediatric in much discussion in audiologist in appropriate identification the audiology profession. and management of infants and children Rapin and Gravel (2003) with this disorder. suggested “auditory neuropathy” is an Approximately 40% of ANSD cases have inappropriate term to use a genetic basis. Manchaiah and colleagues to describe pathologies (2011) found the largest proportion of affecting the central ANSD cases was due to syndromic, non- auditory pathway and syndromic, or mitochondrial genetic brainstem. They suggest this term is best factors. Inheritance patterns included suited to describe pathology limited to autosomal dominant, autosomal recessive, the spiral ganglion cells or their axons X-linked, and mitochondrial. eBook Chapter 8 • Auditory Neuropathy Spectrum Disorder • 8-1 A RESOURCE GUIDE FOR EARLY HEARING DETECTION & INTERVENTION Mutation to Connexin 26 (GJB2) genes Various types of genetic mutations in accounts for 30-35% of autosomal ANSD result in different pathological recessive non-syndromic deafness. changes in the auditory system (Manchaiah Cheng et al. (2005) studied over 700 et al., 2011). Continued clinical study children attending schools for the deaf of genetic etiologies of ANSD may hold or receiving services for moderate- information to assist in specific aspects of profound hearing loss. Of those children, clinical management of cases. Syndromes 76 tested had present OAE responses, ANSD has been associated with include: suggesting a possible diagnosis of ANSD. No electrophysiological tests were • Charcot-Marie Tooth Disease (see conducted. Five of these children had Figure 1) GJB2 mutations. In a study by Santarelli • Leber’s Hereditary Optic Neuropathy et al. (2008), three children with GJB2 • Fredreich’s Ataxia mutations had abnormal audiological • Mohr-Tranabjaerg Syndrome and electrophysiological findings with • Refsum’s Disease preserved OHC functioning—confirming • Mitochondrial disease ANSD. The OTOF gene (otoferlin) has been Various types of genetic widely discussed in cases of ANSD. OTOF Figure 1 mutations in ANSD encodes for otoferlin, which is expressed Extremities of result in different in the cochlea and vestibule, in cochlear Patient with Charcot pathological changes and vestibular nuclei, hippocampus, cerebellum, and testis. In adult cochleae, it in the auditory system. is expressed only in inner hair cells at the Marie Tooth Disease basolateral region, where afferent synaptic contacts are located (Zadro et al., 2010). Pejvakin is a protein detected in the cell bodies of neurons of the afferent auditory pathway. It has been detected in some cases of autosomal recessive ANSD. DFNB59 encodes pejvakin and has shown to cause neural dysfunction along the auditory pathway in humans. Pejvakin is a paralog of DFNA5, which is also a protein involved in deafness (Delmaghani et al., 2006). The MPZ gene (myelin protein zero) was the first gene associated with ANSD in patients with Charcot Marie Tooth Disease. MPZ encodes a protein included Risk Factors in the compact myelin that plays a crucial role in myelin formation and adhesion. A postmortem examination of one patient Risk factors which may contribute to with this etiology of ANSD revealed ANSD include: preserved cochlear hair cells, decreased • Neonatal anoxia spiral ganglion cell number, and extensive • Neonatal hyperbilirubinemia degeneration of both peripheral and • Neonatal mechanical ventilation, central processes in the residual axons. hypoxia, or both The proximal portion of the auditory • Congenital brain abnormalities nerve showed axonal loss and incomplete • Low birthweight remyelination at the entrance to the • Extreme prematurity (< 28 weeks) brainstem (Santarelli, 2010). • Genetics or family history of ANSD eBook Chapter 8 • Auditory Neuropathy Spectrum Disorder • 8-2 NATIONAL CENTER FOR HEARING ASSESSMENT & MANAGEMENT A higher risk of ANSD diagnosis is pediatric audiologist for comprehensive noted for Neonatal Intensive Care Unit testing, which should include the (NICU) graduates (see Figure 2). The Joint following (see Table 1): Commission on Infant Hearing (JCIH, 2007) recommends auditory brainstem • Auditory Brainstem Response (ABR) response (ABR) as part of the screening Testing protocol for NICU babies admitted for • Case History greater than 5 days. NICU infants who do • Otoscopy not pass the automated ABR screen should • Imittance be referred directly to an audiologist for • Otoacoustic Emissions (OAEs) rescreen or comprehensive testing. • Behavioral Audiometry Figure 2 Case Management Children with ANSD should be managed Low Birthweight and by a multidisciplinary team, which in Extreme Prematurity— addition to the pediatric audiologist includes a speech/language pathologist, Known Risk Factors teacher of the deaf and hard of hearing, otolaryngologist, geneticist, neurologist, for ANSD pediatrician, and when necessary, physical and occupational therapists. Management must be considered on an individual basis—considering the unique abilities of each patient. Berlin recommends evaluating language growth and development every 3 months. If the patient does not make progress in language development, management and habilitation programs should be considered.1 A co-morbidity rate of 54% in ANSD patients has been reported in the form of developmental delays, learning delays, attention deficit disorder (ADD), attention deficit hyperactivity disorder (ADHD), autism spectrum disorders, emotional and/or behavioral problems, uncorrected The “1-3-6 rule” of screening for hearing visual problems, blindness, cerebral loss by 1 month of age, confirmation of palsy, motor disorders, apraxia, inner ear presence of hearing loss by 3 months malformation, atretic or absent auditory of age, and intervention by 6 nerve, seizures, and various syndromes.1 months of age is the goal of early These co-morbidities may contribute to detection and intervention of speech/language and learning outcomes. hearing impairment programs.3 A diagnostic test battery of In patients with lack of speech/language immittance, otoacoustic emissions, progress, some benefit from amplification. and threshold ABR testing will Rance et al. (2002) compared unaided correctly diagnose cases of ANSD. and aided speech perception assessments and cortical event-related potentials from Diagnostic Evaluation 18 children diagnosed with ANSD and found that approximately half showed Infants and children with suspected a significant improvement in open- hearing loss should be referred to a set speech perception ability. Of these eBook Chapter 8 • Auditory Neuropathy Spectrum Disorder • 8-3 A RESOURCE GUIDE FOR EARLY HEARING DETECTION & INTERVENTION Table 1 Pediatric Audiologist Testing Auditory Brainstem Response (ABR) Testing ABR testing using insert earphones curve to allow for it to be clamped delivery for patients with ANSD click stimuli in alternating polarities (see Figure 4). Additional runs (either and normal hearing subjects. No at high-intensity levels (condensation rarefaction or condensation polarity) significant differences were noted and rarefaction, 80 or 90dBnHL) to should then be made at the same in patients with ANSD and absent look for the cochlear microphonic high-intensity stimulus levels. If DPOAEs when compared to patients (CM; see Figure 3) is essential. Use the potential is clearly eliminated, a with ANSD and present DPOAE of alternating clicks would yield a true CM exists (see Figure 5). If the responses in terms of CM time delay flat line if a genuine CM response measured potential remains, it is due to latency. CM amplitudes in patients existed (due to cancellation of the a stimulus artifact. The transducer and with ANSD and absent DPOAEs response). In patients with middle electrodes should be separated as much were significantly lower than those ear dysfunction and abnormal as possible, and retesting should be in patients with ANSD and present tympanometry, the CM response
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