The role of electrocochleography and the caloric test in predicting short-term recurrence of benign paroxysmal positional vertigo.

Objective: The study aimed to assess the value of physiological tests for evaluating inner ear function in predicting the short-term recurrence of benign paroxysmal positional vertigo (BPPV). Materials and methods: The clinical information of all idiopathic BPPV patients who were treated in our clinic between February 2021 and December 2022 were reviewed. All patients included in the study had completed audiology examinations including pure tone audiometry, electrocochleography (EcochG), auditory brainstem response, and vestibular function examination such as the vestibular caloric test. The relationships between the results of the above tests and short-term recurrent BPPV were analyzed. Results: A total of 96 patients with unilateral idiopathic BPPV were included for analysis. The numbers of non-recurrent patients and recurrent patients were 57 (59.4%) and 39 (40.6%), respectively. Only the results of EcochG and the caloric test showed significant differences between non-recurrent and recurrent patients (both P < 0.05). The results of these two tests were also found to be independently predictive of short-term recurrence (both P < 0.05). The non-recurrence rate for patients with normal results in both tests reached up to 78.3%, which was significantly higher than that for patients with abnormal results in both tests, 28.6% (P < 0.05). Conclusion: Endolymphatic hydrops and canal paresis were independent risk factors for short-term recurrent BPPV. Additional treatments should be considered to reduce the recurrence rate, including dehydration treatment and vestibular rehabilitation.

Delayed progressive sensorineural hearing loss due to a novel compound heterozygous PTPRQ mutation in a Chinese patient.

BACKGROUND: The Protein tyrosine phosphatase receptor Q (PTPRQ) gene encodes a member of the type III receptor-like protein tyrosine phosphatase family found in the stereocilium. Mutations in PTPRQ are mostly associated with deafness, autosomal recessive type 84 (DFNB 84), which usually results in progressive familial hearing loss. METHODS: A 25-year-old woman and her sister, both with postlingual-delayed progressive sensorineural hearing loss, were examined. They were from a nonconsanguineous marriage and had no family history of hearing loss. New compound heterozygous PTPRQ gene mutations, nonsense (c.90C > A, p.Y30X) and splice (c.5426 + 1G > A) mutations in two PTPRQ alleles, were identified in the two sisters and were presumably autosomal recessive. The c.90C > A (p.Y30X) mutation was mapped to exon 2 of PTPRQ (NM_001145026). RESULTS: The c.90C > A mutation leads to a premature stop codon and a truncated protein. The c.5426 + 1G > A mutation leads to a truncated protein lacking the extracellular domain. Hence, both mutations were predicted to be pathogenic, leading to a deficiency of the extracellular, transmembrane, and phosphatase domains because of nonsense-mediated mRNA degradation. CONCLUSIONS: This study increases the spectrum of PTPRQ gene mutations that might be involved in delayed progressive autosomal recessive non-syndromic hearing loss.

Clinical anatomy of superior laryngeal artery via transoral approach.

Objective: Hemorrhage is the most common complication caused by transoral laryngopharyngeal surgery. It is believed that proper management of the superior laryngeal artery (SLA), the main feeding artery for the larynx and pharynx, may reduce intra- and postoperative hemorrhage incidence. The aim of this study was to illustrate the anatomy of the SLA via transoral endoscopic approach. Methods: Fourteen sides of SLA from heads of seven fresh-frozen and silicone-injected cadavers were dissected. Transoral dissections were performed for the intra-laryngeal segment of SLA, and transcervical dissections were performed to confirm the anatomical measurements. Results: SLA had a slightly descending course from the origin to the larynx, and there was a major branch supplying the epiglottis, named pharyngo-epiglottic artery (PEA). Parallel with the internal superior laryngeal nerve (ISLN), SLA passed through the thyrohyoid membrane and ended into the hypopharynx. The distance from SLA to the superior horn of thyroid cartilage (SHTC) was (9.11 ± 0.58)mm on the left and (9.01 ± 0.37)mm on the right; the distance from SLA to the inferior margin of the hyoid bone (IMHB) was (2.00 ± 0.11)mm on the left and (1.95 ± 0.08)mm on the right; the distance from SLA to ISLN was (5.98 ± 0.48)mm on the left and (5.78 ± 0.36)mm on the right. No significant difference was found between bilateral sides (p > 0.05). Moreover, the distance from SLA to superior margin of thyroid cartilage (SMTC) was (5.52 ± 0.24)mm on the left and (5.80 ± 0.15)mm on the right. A significant difference was also found between bilateral sides (p = 0.03), which might suggest the SLA is located further from the SMTC on the right side. Conclusion: SHTC, SMTC, and IMHB could be regarded as anatomical landmarks to locate SLA when applying a transoral approach. Moreover, a complete understanding of the detailed anatomy of the superior laryngeal artery may improve the detection of hemostasis in transoral laryngeal or hypo-pharyngeal surgery.

Sequential Bilateral Cochlear Implantation With Prolonged Time Intervals.

Purpose The aim of the study was to assess whether sequential cochlear implantation (CI) with a prolonged interimplant interval (M = 15.2 years) between the first and second CIs benefited speech recognition and health-related quality of life. Method This prospective study included 14 prelingually deafened participants who received their second CI after a prolonged interimplant interval (M = 15.2 years). Additionally, speech recognition ability over a 12-month period of bilateral implant use was investigated. The results of the speech recognition test in both quiet and noisy conditions were statistically analyzed for each CI alone and both CIs together. Nijmegen Cochlear Implant Questionnaire scores were also collected at activation and at 12 months after activation. Results Improvements in speech recognition ability were observed following the use of the first implant alone and with the use of both implants together; however, progress was much slower with the use of the second implant alone, following its introduction. Furthermore, a significant difference in the trajectory of speech recognition ability was observed between the first and the second implanted ear. According to Nijmegen Cochlear Implant Questionnaire scores, all participants benefitted from bilateral CI after 12 months. Conclusions Prolonged interimplant intervals resulted in asymmetrical speech recognition abilities. A significant improvement in the speech recognition scores was observed with the first implanted ear, and much slower progress was observed with the second implanted ear. However, the "poorer" second implanted ear could provide a considerable beneficial effect on the improved speech recognition and health-related quality of life with the bilateral CI. Supplemental Material https://doi.org/10.23641/asha.12861152.

Whole exome sequencing identifies SCD5 as a novel causative gene for autosomal dominant nonsyndromic deafness.

We report a genetic assessment of autosomal dominant, nonsyndromic, progressive sensorineural hearing loss in a Chinese family, combining whole-exome sequencing and genome-wide linkage analysis. A novel missense mutation, c.626G > C, in the SCD5 gene was identified in this family. The heterozygous missense mutation could segregate hearing loss cases among family members, and was predicted to be deleterious by Polyphen-2, LRT and Mutation Taster. SCD5 is an endoplasmic reticulum enzyme, catalyzing the formation of monounsaturated fatty acids (MUFAs) from saturated fatty acids (SFAs). It plays a crucial role in regulating lipid metabolism. The SCD5 protein is expressed in inner and outer hair cells of the organ of Corti, the stria vascularis, cells of the lateral cochlear wall behind the spiral prominence, and more strongly in spiral ganglion cells of guinea pig and human fetal cochleas. SCD5 protein was also expressed in the brain, consistent with the hearing loss feature: the patients had a poor speech discrimination score at young age and mild hearing loss as evaluated by pure tone audiometry. In summary, we identified SCD5 as a novel gene responsible for autosomal dominant nonsyndromic deafness.

[Analyses prognostic factors relevant to sudden sensorineural hearing loss].

OBJECTIVE: To investigate the prognostic factors relevant to sudden sensorineural hearing loss. METHODS: The internationally accepted standardized clinical research methods, unified design, and unified program were adopted to conduct the prospective clinical multi-center study. The sudden deafness patients between 18 to 65 years old, with the course of this disorder less than two weeks, and without any medical treatments were collected, and then, divided into four types according to the hearing curve: type A, acute sensorineural hearing loss in low tone frequencies; type B, acute sensorineural hearing loss in high tone frequencies; type C, acute sensorineural hearing loss in all frequencies; and type D, total deafness. The factors, in terms of age, gender, type of initial audiogram, time delay before the first visit, and severity of hearing loss, were included in the analyses. RESULTS: A total of 1 024 cases with single side sudden deafness were collected in the study from 33 hospitals in China from August 2007 to October 2011, inclusive of for 492 males (48.05%) and 532 females (51.95%). The average age was (41.2 ± 12.8) years old. There were 553 cases (54.00%) in left ear, and 471 cases (46.00%) in right ear. The curative effects of different types were shown as follows: the type in low tone frequencies had the highest rate of 90.73%, the type in all frequencies was 82.59%; the type of total deafness was 70.29%; and the type in high tone frequencies had the lowest rate of 65.96%. It had significant difference of the effective rate between different types (χ(2) = 231.58, P = 0.000). Age, time delay before first visit, and severity of initial hearing loss were significantly correlated with hearing improvement. CONCLUSIONS: Initial audiogram of SSNHL might predict hearing recovery. The young in age and a short time delay before starting treatment are positive prognostic factors for hearing recovery in SSNHL. The initial severity of hearing loss is negative prognostic factor of hearing recovery.

[Surgical treatment for advanced carcinoma of hypopharynx and laryngeal function preservation].

OBJECTIVE: To evaluate the treatment outcome advanced hypopharyngeal carcinoma by surgery with laryngeal function preservation. METHODS: Twenty cases with squamous cell carcinoma of the hypopharynx who were treated by the function preserved surgery between January 2000 and March 2012 were reviewed. Of the patients 19 were males and 1 female, and their median age was 62.0 years. Eight cases were only applied with total or subtotal hypopharyngectomy, and others received total or subtotal hypopharyngectomy with partial-laryngectomy. Postoperative functional training was performed. Radiotherapy was used in all cases from 2 to 4 weeks after surgery. RESULTS: Speech intelligibility remained in all 20 patients and 80.0% of the patients were extracted the trachea tube in the average 60 days. CONCLUSIONS: Laryngeal functions of larynx can be preserved in advanced hypopharyngeal carcinoma by total or subtotal hypopharyngectomy alone or plus partial laryngectomy with functional reconstruction, and the patients can maintain their rational speech and swallowing functions.