Auditory neuropathy spectrum disorder: a review.

PURPOSE: Auditory neuropathy spectrum disorder, or ANSD, can be a confusing diagnosis to physicians, clinicians, those diagnosed, and parents of children diagnosed with the condition. The purpose of this review is to provide the reader with an understanding of the disorder, the limitations in current tools to determine site(s) of lesion, and management techniques. METHOD: This article is a review of what is known about ANSD. It includes descriptions of assessment tools, causes of ANSD, and patient management techniques. CONCLUSIONS: This review is a guide to audiologists, speech-language pathologists, and early interventionists who work with individuals diagnosed with ANSD and/or their families. It highlights the need for more precise tools to describe the disorder in order to facilitate decisions about interventions and lead to better predictions of outcome.

Interaural multiple frequency tympanometry measures: clinical utility for unilateral conductive hearing loss.

BACKGROUND: Tympanometry is a routine clinical measurement of the acoustic immittance of the ear as a function of ear canal air pressure. The 226 Hz tympanogram can provide clinical evidence for conditions such as a tympanic membrane perforation, Eustachian tube dysfunction, middle ear fluid, and ossicular discontinuity. Multiple frequency tympanometry using a range of probe tone frequencies from low to high has been shown to be more sensitive than a single probe tone tympanogram in distinguishing between mass- and stiffness-related middle ear pathologies (Colletti, 1975; Funasaka et al, 1984; Van Camp et al, 1986). PURPOSE: In this study we obtained normative measures of middle ear resonance by using multiple probe tone frequency tympanometry. Ninety percent ranges for middle ear resonance and for interaural differences were calculated. RESEARCH DESIGN: In a mixed design, normative data were collected from both ears of male and female adults. STUDY SAMPLE: Twelve male and 12 female adults with normal hearing and normal middle ear function participated in the study. DATA COLLECTION AND ANALYSIS: Multiple frequency tympanograms were recorded with a commercially available immittance instrument (GSI Tympstar) to obtain estimates of middle ear resonant frequency (RF) using ΔB, positive tail, and negative tail methods. Data were analyzed using three-way mixed analyses of variance with gender as a between-subject variable and ear and method as within-subject variables. T-tests were performed, using the Bonferroni adjustment, to determine significant differences between means. RESULTS: Using the positive and negative tail methods, a wide range of approximately 500 Hz was found for middle ear resonance in adults with normal hearing and normal middle ear function. The difference in RF between an individual's ears is small with 90% ranges of approximately ±200 Hz, indicating that the right ear RF should be either 200 Hz higher or lower in frequency compared to the left ear. This was true for both negative and positive tail methods. CONCLUSION: Ninety percent ranges were calculated to determine the difference in middle ear resonance expected between an individual's ears. These ranges can provide critical normative values for determining how pathology in an ear with a unilateral conductive hearing loss is altering the mass or stiffness characteristics of the middle ear system.

Tectorial membrane morphological variation: effects upon stimulus frequency otoacoustic emissions.

The tectorial membrane (TM) is widely believed to play an important role in determining the ear's ability to detect and resolve incoming acoustic information. While it is still unclear precisely what that role is, the TM has been hypothesized to help overcome viscous forces and thereby sharpen mechanical tuning of the sensory cells. Lizards present a unique opportunity to further study the role of the TM given the diverse inner-ear morphological differences across species. Furthermore, stimulus-frequency otoacoustic emissions (SFOAEs), sounds emitted by the ear in response to a tone, noninvasively probe the frequency selectivity of the ear. We report estimates of auditory tuning derived from SFOAEs for 12 different species of lizards with widely varying TM morphology. Despite gross anatomical differences across the species examined herein, low-level SFOAEs were readily measurable in all ears tested, even in non-TM species whose basilar papilla contained as few as 50-60 hair cells. Our measurements generally support theoretical predictions: longer delays/sharper tuning features are found in species with a TM relative to those without. However, SFOAEs from at least one non-TM species (Anolis) with long delays suggest there are likely additional micromechanical factors at play that can directly affect tuning. Additionally, in the one species examined with a continuous TM (Aspidoscelis) where cell-to-cell coupling is presumably relatively stronger, delays were intermediate. This observation appears consistent with recent reports that suggest the TM may play a more complex macromechanical role in the mammalian cochlea via longitudinal energy distribution (and thereby affect tuning). Although significant differences exist between reptilian and mammalian auditory biophysics, understanding lizard OAE generation mechanisms yields significant insight into fundamental principles at work in all vertebrate ears.

Prevalence of stereotypical responses to mistuned complex tones in the inferior colliculus.

The human auditory system has an exceptional ability to separate competing sounds, but the neural mechanisms that underlie this ability are not understood. Responses of inferior colliculus (IC) neurons to "mistuned" complex tones were measured to investigate possible neural mechanisms for spectral segregation. A mistuned tone is a harmonic complex tone in which the frequency of one component has been changed; that component may be heard as a separate sound source, suggesting that the mistuned tone engages the same mechanisms that contribute to the segregation of natural sounds. In this study, the harmonic tone consisted of eight harmonics of 250 Hz; in the mistuned tone, the frequency of the fourth harmonic was increased by 12% (120 Hz). The mistuned tone elicited a stereotypical discharge pattern, consisting of peaks separated by about 8 ms and a response envelope modulated with a period of 100 ms, which bore little resemblance to the discharge pattern elicited by the harmonic tone or to the stimulus waveform. Similar discharge patterns were elicited from many neurons with a range of characteristic frequencies, especially from neurons that exhibited short-latency sustained responses to pure tones. In contrast, transient and long-latency neurons usually did not exhibit the stereotypical discharge pattern. The discharge pattern was generally stable when the stimulus level or component phase was varied; the major effect of these manipulations was to shift the phase of the response envelope. Simulation of IC responses with a computational model suggested that off-frequency inhibition could produce discharge patterns with these characteristics.

Dendritic orientation and laminar architecture in the rabbit auditory thalamus.

A laminar organization composed of the dendritic fields of principal neurons and afferent axonal arbors has been proposed as the anatomical substrate for the frequency map at several levels of the mammalian central auditory system, including the inferior colliculus and medial geniculate body (MGB). In contrast to the auditory thalamus in most mammals, the ventral division of the rabbit medial geniculate body (MGV) has cellular laminae visible in routine Nissl stains, allowing a direct comparison of the laminar organization with the dendritic architecture and frequency organization. In total 30 presumptive relay neurons in the MGV were labeled with the juxtacellular recording method, and their dendritic arbors were fully reconstructed from serial sections with the aid of a computer microscope. The spatial organization of MGV dendritic fields was analyzed using the dendritic prism, dendritic stick, and fan-in projection methods. Quantitative spatial analyses revealed that, for MGV neurons in the central pars lateralis subdivision, the major axis of the dendritic fields (approximately 29 degrees relative to the horizontal plane) was closely aligned with that of the Nissl laminae (approximately 25 degrees). Both were oriented orthogonally to the tonotopic axis. In contrast, cells in the pars ovoidea had their major axis of orientation parallel to the anteroposterior axis of the brain. Although a bitufted dendritic field was the norm, it was not uncommon for MGV neurons to have pronounced spatial asymmetries in their dendritic fields. A model is presented that incorporates cellular laminae and oriented dendritic growth to form frequency-related slabs within the MGV.

Functional subregions in primary auditory cortex defined by thalamocortical terminal arbors: an electrophysiological and anterograde labeling study.

Several functional maps have been described in primary auditory cortex, including those related to frequency, tuning, latency, binaurality, and intensity. Many of these maps are arranged in a discontinuous or patchy manner. Similarly, thalamocortical projections arising from the ventral division of the medial geniculate body to the primary auditory cortex are also patchy. We used anterograde labeling and electrophysiological methods to examine the relationship between thalamocortical patches and auditory cortical maps. Biotinylated dextran-amine was deposited into physiologically characterized sites in the ventral division of the medial geniculate body of New Zealand white rabbits. Approximately 7 d later, the animal was again anesthetized and the ipsilateral auditory cortex was mapped with tungsten microelectrodes. Multi-unit physiological data were obtained for the following characteristics: best frequency (BF), binaurality, response type, latency, sharpness of tuning, and threshold. Immunocytochemical methods were used to reveal the injection site in the ventral division of the medial geniculate body as well as the anterogradely labeled thalamocortical afferents in the auditory cortex. In 86% of the cases (12 of 14), entry into a thalamocortical patch was associated with a marked change in physiological responses. A consistent BF and binaural class were usually observed within a patch. The patches appear to innervate distinct functional regions coding frequency and binaurality. A model is presented showing how patchy thalamocortical projections participate in the formation of tonotopic and binaural maps in primary auditory cortex.

Cell types and response properties of neurons in the ventral division of the medial geniculate body of the rabbit.

Although there is evidence for multiple classes of thalamic relay neurons in the auditory thalamus, correlative anatomical and physiological studies are lacking. We have used the juxtacellular labeling technique, in conjunction with Nissl, Golgi, and immunocytochemical methods, to study the morphology and response properties of cells in the ventral division of the medial geniculate body of the rabbit. Single units in the ventral division of the medial geniculate body (MGV) were characterized extracellularly with monaural and binaural tone and noise bursts (100- to 250-msec duration). Characterized units were filled with biocytin and visualized with an antibody enhanced diaminobenzidine reaction. A total of 31 neurons were physiologically characterized and labeled with the juxtacellular technique. Labeled neurons were fully reconstructed from serial sections by using a computer microscope system. Three subregions of the rabbit MGV were identified, each characterized by differences in Nissl architecture, calcium-binding protein expression, and by the dendritic orientation of tufted relay neurons. In general, the dendritic fields of relay neurons were closely aligned with the cellular laminae. Qualitative and quantitative analyses revealed two types of presumptive relay neurons within the MGV. Type I cells had thick dendrites with a greater total volume and morphologically diverse appendages compared with the Type II cells whose dendrites were thin with a moderate number of small spines. Both classes were acoustically responsive and exhibited a variety of response patterns, including onset, offset, and sustained responses. In terms of binaural characteristics, most (ca. 53%) labeled neurons were of the EE type, with the remaining cells classified as EO (27%) or EI (20%) response types. Two types of presumptive interneurons were also seen: bipolar neurons with large dendritic fields and a small neurogliaform variety. Cell types and dendritic orientation within the MGV are discussed in terms of the physiological organization of the rabbit auditory thalamus.