Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic.

UNLABELLED: Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency. CONCLUSION: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients. WHAT IS KNOWN: • Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates. • Little data are available on the prevalence and risk factors for ANSD in healthy newborns. What is new: • The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births. • In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.

Hearing performance in single-sided deaf cochlear implant users after upgrade to a single-unit speech processor.

INTRODUCTION: Single-sided deaf (SSD) patients report multiple benefits after cochlear implantation (CI), such as tinnitus suppression, speech perception, and sound localization. The first single-unit speech processor, the RONDO, was launched recently. Both the RONDO and the well-known behind-the-ear (BTE) speech processor work on the same audio processor platform. However, in contrast to the BTE, the microphone placement on the RONDO is different. The aim of this study was to evaluate the hearing performances using the BTE speech processor versus using the single-unit speech processor. Subjective and objective outcomes in SSD CI patients with a BTE speech processor and a single-unit speech processor, with particular focus on spatial hearing, were compared. METHODOLOGY: Ten adults with unilateral incapacitating tinnitus resulting from ipsilateral sensorineural deafness were enrolled in the study. The mean age at enrollment in the study was 56 (standard deviation, 13) years. The subjects were cochlear implanted at a mean age of 48 (standard deviation, 14) years and had on average 8 years' experience with their CI (range, 4-11 yr). At the first test interval (T0), testing was conducted using the subject's BTE speech processor, with which they were already familiar. Aided free-field audiometry, speech reception in noise, and sound localization testing were performed. Self-administered questionnaires on subjective evaluation consisted of HISQUI-NL, SSQ5, SHQ, and a Visual Analogue Scale to assess tinnitus loudness and disturbance. All 10 subjects were upgraded to the single-unit processor and retested after 28 days (T28) with the same fitting map. At T28, an additional single-unit questionnaire was administered to determine qualitative experiences and the effect of the position of the microphone on the new speech processor. RESULTS: Equal hearing outcomes were found between the single-unit speech processor: median PTA(single-unit) (0.5, 1, 2 kHz) = 40 (range, 33-48) dB HL; median Speech Reception Threshold in noise = -1.00 (range, -8.50 to +1.00) dB SNR; median Root Mean Square Error of sound localization = 45 (range, 19-139) degrees; HISQUI = 128 (range, 106-180); SHQ = 68 (range, 45-83); SSQ5 = 6 (range, 3-9) and the BTE speech processor: median PTA(BTE) (0.5, 1, 2 kHz) = 41 (range, 30-53) dB HL; median Speech Reception Threshold in noise = -0.25 (range, -7.00 to +4.00) dB SNR; median Root Mean Square Error of sound localization = 38 (range, 26-164) degrees; HISQUI = 144 (range, 120-183); SHQ = 56 (range, 47-85); SSQ5 = 6 (range, 3-9). The results in the condition with the single-unit speech processor were not significantly influenced by the position of the microphone. CONCLUSION: The study showed that long-term BTE speech processor SSD users are able to be upgraded to a single-unit speech processor without compromising their speech performance, aided hearing thresholds, sound localization, objective speech quality, hearing abilities, sound localization, and tinnitus reduction. Microphone position on the single-unit speech processor did not influence the outcomes measures. Moreover, after a short time of experience, 80% of the users preferred the single-unit processor.

Clinical need for a Baha trial in patients with single-sided sensorineural deafness. Analysis of a Baha database of 196 patients.

A bone-anchored hearing aid (Baha) is used in patients with single-sided sensorineural deafness (SSD) to overcome the head shadow effect. Of all the patients with SSD, treated at our hospital, 196 patients used a Baha on trial between November 2001 and April 2010. The objective of this study is to evaluate what factors determine the decision of a SSD patient whether or not to opt for a Baha device following a Baha trial period. 196 patients with SSD were enrolled for a trial period of 2 weeks at the Antwerp University Hospital, a tertiary referral centre. 93% of these patients suffered from an acquired hearing loss. 44% of all the patients (87/196) chose to wear a Baha device after the trail period, either on an abutment or on a headband. The collected data were analysed to determine correlations between the decision of a patient following a Baha trial period on the one hand, and Fletcher Index ipsi- and contralaterally, bone conduction hearing thresholds at the better hearing ear, aetiology, age at the start of the trial period, duration of hearing loss at the start of the trial period and the type of device used during the trial period, on the other hand. Although 66% of all the patients (109/196) declined the Baha after a trial, reasons not to choose a Baha were diverse and no crucial factors could be found that determine the success of a Baha trial period. Lack of improvement concerning speech understanding in noise was the most important reason mentioned by patients who declined the Baha. The authors advocate that all patients, suffering from SSD, should be offered the opportunity to try a Baha device as no factors could be found that determine the decision of a patient following the trial period.

Cochlear implantation as a durable tinnitus treatment in single-sided deafness.

INTRODUCTION: Severe tinnitus can seriously impair patients in their activities in daily life and reduce their quality of life. The aims of this prospective clinical study were to assess the long-term effects of cochlear implantation (CI) on tinnitus in patients with single-sided deafness and ipsilateral incapacitating tinnitus, and to investigate whether CI could treat various types of tinnitus. MATERIALS AND METHODS: Twenty-six subjects with unilateral severe-to-profound sensorineural hearing loss received a CI. Patients suffered from severe tinnitus greater than 6/10 on a visual analogue scale (VAS) due to unilateral deafness. Assessment consisted of a tinnitus analysis including determination of tinnitus type, frequency, and loudness. A tinnitus questionnaire (TQ) measured tinnitus distress. VAS and TQ were administered pre-implantation and post-implantation. RESULTS: All 26 patients reported a subjective benefit from CI. Tinnitus loudness reduced significantly after CI from 8.6 to 2.2 on the VAS (scale: 0-10). The TQ total score decreased significantly and the mean tinnitus degree decreased from severe to mild. No differences were observed between patients with pure-tone tinnitus, narrow band noise tinnitus, or polyphonic tinnitus. The degree of tinnitus loudness reduction remained stable after CI. CONCLUSIONS: CI can successfully be used as treatment of severe tinnitus in patients with single-sided deafness and is equally effective for pure tone, narrow band noise, and polyphonic tinnitus. Long-term results show that implantation provides durable tinnitus relief in these patients. These results support the hypothesis that physiopathological mechanisms after peripheral deafferentation are reversible when hearing is restored. Single-sided deafness accompanied by severe tinnitus is a new indication for CI.

Quality-of-life benefit from cochlear implantation in the elderly.

OBJECTIVE: To compare the audiologic results of geriatric patients receiving cochlear implants with younger age groups and to evaluate the quality of life after cochlear implantation in the geriatric population by means of validated quality-of-life questionnaires. STUDY DESIGN: Cross-sectional study involving 89 postlingually deafened cochlear implant subjects. SETTING: Tertiary referral center. PATIENTS: A total of 89 postlingually deafened patients were included in the study, among which were 25 patients who were aged 70 years or older. INTERVENTIONS: All patients received a cochlear implant. Subjects were implanted with either the Laura, Nucleus 24, or Med-el Combi 40+ cochlear implant systems implementing the SPEAK, ACE, CIS, or CIS+ coding strategies. MEAN OUTCOME MEASURES: Speech recognition was determined by means of phonetically balanced monosyllabic word lists. The Hearing Handicap Inventory for Adults, the Glasgow Benefit Inventory, and the scale for the prediction of hearing disability in sensorineural hearing loss were used to quantify the quality of life. RESULTS: Mean audiologic performance for the three groups increased significantly after implantation (p < 0.001). Postoperative audiologic performance of the geriatric population led to useful hearing, but these scores were significantly lower than for the younger age groups (p = 0.002). However, the quality-of-life outcomes for the geriatric group were similar to those of the younger age groups (p = 0.411 for the Hearing Handicap Inventory for Adults; p = 0.886 for the Glasgow Benefit Inventory). CONCLUSION: The results of this study prove that cochlear implantation in the elderly provides improvements in quality of life and speech understanding, similar to those for younger adult cochlear implant recipients.