Auditory dysfunction in type 2 Stickler Syndrome.

PURPOSE: To present the extent and site of lesion of auditory dysfunction in a large cohort of individuals with type 2 Stickler Syndrome. Type 2 Stickler Syndrome results from a mutation in the gene coding for α-1 type XI pro-collagen, which has been identified in the human vitreous, cartilage and the cochlea of the mouse. The condition is characterised by classic ocular abnormalities, auditory dysfunction, osteoarthropathy and oro-facial dysplasia. METHODS: This is a population study which used a combination of audiometric, tympanometric, and self-report measures on a series of 65 individuals (mean age 29.2 years, range 3-70, female 63.1%) with genetically confirmed type 2 Stickler Syndrome. RESULTS: Hearing impairment was identified in at least one ear for 69% of individuals. Analysis against age-matched normative data showed that reduced hearing sensitivity was present across all test frequencies. Sensorineural hearing loss was most common (77% of ears), with conductive (3%), mixed (7%) and no hearing loss (13%), respectively. The proportion of hypermobile tympanic membranes (24%) was less than previously documented in type 1 Stickler Syndrome. When present, this appears to arise as a direct result of collagen abnormalities in the middle ear. Self-report measures of speech and spatial hearing in sound were comparable to a non-syndromic cohort with similar audiometric thresholds. CONCLUSIONS: Auditory impairment in type 2 Stickler Syndrome is predominantly associated with cochlear hearing loss of varying severities across affected individuals. The impact on hearing thresholds can be seen across the frequency range, suggesting a contribution of defective collagen throughout the cochlea. Self-report questionnaires showed that difficulties understanding speech, and spatial information in sound (such as that used for localisation), were worse than a young, normal-hearing population but comparable to a non-syndromic cohort with similar audiometric thresholds. Therefore, it is likely that hearing loss in type 2 Stickler Syndrome arises in the auditory periphery, without significant central processing deficits.

A Cross-Sectional Questionnaire Study of Tinnitus Awareness and Impact in a Population of Adult Cochlear Implant Users.

OBJECTIVES: The primary aim was to identify the proportion of individuals within the adult cochlear implant population who are aware of tinnitus and those who report a negative impact from this perception, using a bespoke questionnaire designed to limit bias. A secondary aim was to use qualitative analysis of open-text responses to identify themes linked to tinnitus perception in this population. DESIGN: A cross-sectional questionnaire study of a large clinical population who received an implant from Cambridge University Hospitals, United Kingdom. RESULTS: Seventy-five percent of respondents reported tinnitus awareness. When impact scores for six areas of difficulty were ranked, 13% of individuals ranked tinnitus their primary concern and nearly a third ranked tinnitus in the top two positions. Tinnitus impact was not found to reduce with duration since implantation. The most common open-text responses were linked to a general improvement postimplantation and acute tinnitus alleviation specific to times when the device was in use. CONCLUSIONS: Tinnitus is a problem for a significant proportion of individuals with a cochlear implant. Clinicians, scientists, and cochlear implant manufacturers should be aware that management of tinnitus may be a greater priority for an implantee than difficulties linked to speech perception. Where a positive effect of implantation was reported, there was greater evidence for masking of tinnitus via the implant rather than reversal of maladaptive plasticity.

Perception of stochastic envelopes by normal-hearing and cochlear-implant listeners.

We assessed auditory sensitivity to three classes of temporal-envelope statistics (modulation depth, modulation rate, and comodulation) that are important for the perception of 'sound textures'. The textures were generated by a probabilistic model that prescribes the temporal statistics of a selected number of modulation envelopes, superimposed onto noise carriers. Discrimination thresholds were measured for normal-hearing (NH) listeners and users of a MED-EL pulsar cochlear implant (CI), for separate manipulations of the average rate and modulation depth of the envelope in each frequency band of the stimulus, and of the co-modulation between bands. Normal-hearing (NH) listeners' discrimination of envelope rate was similar for baseline modulation rates of 5 and 34 Hz, and much poorer than previously reported for sinusoidally amplitude-modulated sounds. In contrast, discrimination of model parameters that controlled modulation depth was poorer at the lower baseline rate, consistent with the idea that, at the lower rate, subjects get fewer 'looks' at the relevant information when comparing stimuli differing in modulation depth. NH listeners could discriminate differences in co-modulation across bands; a multidimensional scaling study revealed that this was likely due to genuine across-frequency processing, rather than within-channel cues. CI users' discrimination performance was worse overall than for NH listeners, but showed a similar dependence on stimulus parameters.

Audiologists' preferences for patient-centredness: a cross-sectional questionnaire study of cross-cultural differences and similarities among professionals in Portugal, India and Iran.

OBJECTIVE: Patient-centredness has become an important aspect of health service delivery; however, there are a limited number of studies that focus on this concept in the domain of hearing healthcare. The objective of this study was to examine and compare audiologists' preferences for patient-centredness in Portugal, India and Iran. DESIGN: The study used a cross-sectional survey design with audiologists recruited from three different countries. PARTICIPANTS: A total of 191 fully-completed responses were included in the analysis (55 from Portugal, 78 from India and 58 from Iran). MAIN OUTCOME MEASURE: The Patient-Practitioner Orientation Scale (PPOS). RESULTS: PPOS mean scores suggest that audiologists have a preference for patient-centredness (ie, mean of 3.6 in a 5-point scale). However, marked differences were observed between specific PPOS items suggesting these preferences vary across clinical situations. A significant level of difference (p<0.001) was found between audiologists' preferences for patient-centredness in three countries. Audiologists in Portugal had a greater preference for patient-centredness when compared to audiologists in India and Iran, although no significant differences were found in terms of age and duration of experience among these sample populations. CONCLUSIONS: There are differences and similarities in audiologists' preferences for patient-centredness among countries. These findings may have implications for the training of professionals and also for clinical practice in terms of optimising hearing healthcare across countries.