Preservation of Distortion Product Otoacoustic Emissions in OTOF -Related Hearing Impairment.

OBJECTIVES: Attenuation of otoacoustic emissions over time has been reported for many patients with hearing impairment harboring mutations in the OTOF gene. In this study, the time course of changes of distortion product otoacoustic emissions (DPOAEs) has been analyzed in a cohort of patients in the light of tympanometry results. DESIGN: The changes of DPOAEs in 16 patients with OTOF -related hearing impairment were retrospectively analyzed. RESULTS: All but one subject showed DPOAEs bilaterally at the time of diagnosis. Three patients diagnosed as adults still had DPOAEs at ages of 27, 31, and 47 years, respectively. Follow-up was available for 7 children diagnosed at the age of 1 to 3 years, who still showed preservation of DPOAEs at ages of 5 to 16 years. The responses were absent or attenuated in amplitude at some follow-up appointments in association with type B or C tympanograms. CONCLUSIONS: DPOAEs are preserved much longer than expected in a cohort of patients with OTOF -related hearing impairment. The previously reported loss of DPOAEs may have been caused in some children by increased middle ear impedance due to otitis media.

OTOPLAN in Cochlear Implantation for Far-advanced Otosclerosis.

AIM: To evaluate OTOPLAN software in the surgical planning of far-advanced otosclerosis (FAO) candidates to cochlear implant (CI) were considered. We aimed to preliminarily investigate if this software could influence the electrode length choice, or predict surgical difficulties. METHODS: We reviewed the outcome of five consecutive FAO patients that underwent unilateral CI. OTOPLAN was used to evaluate preoperative computed tomography imaging. A comparison was made with historical data (eight FAO patients). RESULTS: The mean cochlear duct length estimated by OTOPLAN was 32.4 mm. Evaluating OTOPLAN reconstructed images, we ruled out cochlear lumen fibrosis/ossification in three FAO patients and the longest electrodes according to cochlear dimensions were preferred. We disclosed fibrosis in the middle and apical turns of two patients. These findings allowed us to change surgical plans, choosing a shorter electrode (24 and 28 mm instead of 31 mm) to avoid incomplete insertion due to cochlear fibrosis. OTOPLAN reconstructed images identified preoperatively the two patients with round window niche ossification that required additional drilling during surgery. In the present series, we experienced no incomplete insertion. In two cases out of eight historical FAO patients, array insertion (24 mm) was incomplete. One years after unilateral CI, the mean speech reception threshold and disyllabic word recognition score of the five considered FAO patients were 36 dB and 94%, respectively (39 dB and 84% for the eight historical patients). There was no facial nerve stimulation or any other complication during the 1-year follow-up. CONCLUSION: According to our preliminary results, OTOPLAN was useful for the appropriate choice of array length.

A panel of jitter/shimmer may identify functional dysphonia at risk of failure after speech therapy.

BACKGROUND: There are no reliable outcome predictors for functional dysphonia (FD) patients. OBJECTIVES: To investigate if any clinical or phoniatric characteristics could identify FD patients at risk of negative outcome after speech therapy. METHODS: We retrospectively reviewed the results of 78 FD patients treated with the proprioceptive elastic method. Before and one-month after therapy, patients underwent endoscopy, acoustic analysis with Multi-Dimensional Voice Program, and Voice Handicap Index-10 questionnaire (VHI-10). Negative outcome was the persistence of VHI-10 ≥ 13. RESULTS: 26 FD patients had negative outcome (i.e. VHI-10 ≥ 13) after speech therapy. At univariate analysis, clinical variables (i.e. sex, age, comorbidities, dysphonia duration, and professional voice use) were not associated with the outcome. Elevated Jitter% (Jitt; p = 0.03), Shimmer% (Shim; statistical trend, p = 0.06), and Noise to Harmonics Ratio (statistical trend, p = 0.06) were found in patients with poor results. At multivariate analysis, higher Jitt was an independent negative prognostic factor (p = 0.02), while a statically trend was identified for Shim (p = 0.06). A panel of Jitt >1.5 and Shim >5.1 showed an acceptable discriminatory power (AUC [ROC] = 0.76) according to Hosmer and Lemeshow scale. CONCLUSION: A panel of two acoustic analysis parameters could help in identifying FD patients at risk of speech therapy failure. Further studies in these patients are needed to evaluate the most efficient treatment protocol.