Wolfram syndrome (WFS) is an uncommon autosomal recessive neurodegenerative disorder characterized by diabetes mellitus, diabetes insipidus, optic nerve degeneration, hearing impairment, and other abnormalities. Additionally, a portion of individuals experience neurological, endocrine, behavioral, and urinary tract disorders that make management more challenging. Here, we present a 22-year-old male who was diagnosed with type 1 diabetes at the age of 4 and received treatment with basal-bolus insulin therapy. He had blurring of vision and hearing loss at 13 years of age, and our evaluation revealed optic atrophy and sensorineural hearing loss. He had polydipsia and polyuria (intake/output of 5-6 L/day) despite a fairly controlled blood glucose level. Serum anti-diuretic hormone (ADH) was done, which confirmed the diagnosis of central diabetes insipidus. His sonogram and urinary flow studies revealed bilateral hydroureteronephrosis with reflux uropathy. We diagnosed him with neurogenic bladder disorder with detrusor sphincter dyssynergia. This patient had an early onset urological disorder with involvement of eyes and ears, with diabetes mellitus and diabetes insipidus, which satisfied the criteria of WFS. The genetic test confirmed the diagnosis. He is currently being managed with insulin and desmopressin.

OBJECTIVE: Steroids given systemically, locally, or both are the mainstay of treatment for acute acoustic trauma (AAT). The overall recovery rate (full, partial, and none) is undetermined.
METHODS: Original case series and systematic literature review.
METHODS: Case series of a tertiary referral center and a systematic literature review.
METHODS: Cases of AAT between 2012 and 2022 were retrospectively analyzed for demographics, acoustic trauma characteristics, treatment modality and delay and prognosis. This case series was added to the series identified by a systematic literature review. This review included \"Medline\" via \"PubMed\", \"EMBASE\", and \"Google scholar\". All series were pooled for meta-analysis defining prognosis following steroidal treatment for AAT patients.
RESULTS: The pooled analyses included 662 ears, out of which 250 underwent complete recovery of hearing (overall proportion = 0.2809, 95%confidence interval [CI] = 0.1611-0.4178). Any recovery was recorded for 477 ears (overall proportion = 0.7185, 95% CI = 0.5671-0.8493) and no recovery was documented for 185 ears (overall proportion = 0.2815, 95% CI = 0.1507-0.4329).
CONCLUSIONS: The rate of overall recovery for AAT is around 70%, and around 30% for full recovery when steroids are initiated within the first 2 weeks following the insult.
METHODS:

Executive functions (EFs) are related abilities, associated with the frontal lobes functions, that allow individuals to modify behavioral patterns when they become unsatisfactory. The aim of this study was to assess EFs in children with sensorineural hearing loss (SNHL) and in children with \"specific language impairment\" (SLI), compared with a control group of children with normal development, to identify specific skill deficits. Three groups of preschool children aged between 2 and 6 years were assessed: 19 children with normal hearing, cognitive, and language development, 10 children with SNHL, and 20 children with SLI. The FE-PS 2-6 Battery was used for the assessment of preschool EFs, supplemented with the Modified Bell Test for the analysis of selective attention. Statistically significant differences were found between the two experimental groups and the control one, regarding the investigated skills. Children with SNHL showed a clear deficit in flexibility, whereas children with SLI had greater problems in self-regulation and management of waiting for gratification. Selective attention was found to be deficient in all three groups, with no statistically significant differences. This study shows that the skills investigated were found to be deficient in both SNHL and SLI patients. It is essential to start targeted exercises based on specific deficient skills as part of the rehabilitation program. It is of great importance to understand the consequences of EF deficit in preschool children to achieve an accurate diagnosis and carry out customized rehabilitation programs.

Meniere\'s disease is defined by the presence of three essential symptoms: episodic vertigo, tinnitus, and sensorineural hearing loss. The mainstay of its management constitutes lifestyle modification and medical and surgical therapies. Cupping therapy is an ancient treatment that is still widely used especially in the Middle East, Africa, and the United Kingdom. This study portraits the case of a 54-year-old patient suffering from long-standing Meniere\'s disease. The patient was treated with the routine treatment that was to no avail. It was decided that the patient undergoes cupping therapy. Over two years of monthly cupping therapy sessions, the patient reported a decrease in intensity and frequency of symptoms until its disappearance. Cupping therapy has shown a positive outcome on the patient. According to our search, there is a previous case report published in 2020 that shares multiple similarities with our case. Further studies on cupping therapy and its efficacy, mechanism of action, and complications on a larger scale are advised.

There are discrepancies in the literature about the specific influence of leprosy on auditory functions. In routine clinical practice regular hearing screening of leprosy patients is not done. Due to conflicting reports of auditory system involvement in the literature, we conducted this case control study to evaluate the need of regular hearing screening in leprosy patients. A complete otological examination of thirty leprosy patients was conducted. Thirty age and sex matched healthy individual attending ear, nose and throat outpatient department were enrolled as control. Ten cases (33.3%) out of the thirty leprosy patients were found to have high frequency (4 and 8 kHZ) sensorineural hearing loss. The results of this study suggest that leprosy patients require routine monitoring for auditory functions for early identifications of sensorineural hearing loss. As our study is a case-control study with small sample size, in future large prospective studies are required to evaluate the correlation of hearing loss with leprosy and to see the progression hearing loss.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s12070-023-04381-1.

OBJECTIVE: To investigate the assumption that day-case cochlear implantation is associated with lower costs, compared to inpatient cochlear implantation, while maintaining equal quality of life (QoL) and hearing outcomes, for the Dutch healthcare setting.
METHODS: A single-center, non-blinded, randomized controlled trial in a tertiary referral center.
METHODS: Thirty adult patients with post-lingual bilateral sensorineural hearing loss eligible for unilateral cochlear implantation surgery were randomly assigned to either the day-case or inpatient treatment group (i.e., one night admission). We performed an intention-to-treat evaluation of the difference of the total health care-related costs, hospital and out of hospital costs, between day-case and inpatient cochlear implantation, from a hospital and patient perspective over the course of one year. Audiometric outcomes, assessed using CVC scores, and QoL, assessed using the EQ-5D and HUI3 questionnaires, were taken into account.
RESULTS: There were two drop-outs. The total health care-related costs were €41,828 in the inpatient group (n = 14) and €42,710 in the day-case group (n = 14). The mean postoperative hospital stay was 1.2 days (mean costs of €1,069) in the inpatient group and 0.7 days (mean costs of €701) for the day-case group. There were no statistically significant differences in postoperative hospital and out of hospital costs. The QoL at 2 months and 1 year postoperative, measured by the EQ-5D index value and HUI3 showed no statistically significant difference. The EQ-5D VAS score measured at 1 year postoperatively was statistically significantly higher in the inpatient group (84/100) than in the day-case group (65/100). There were no differences in postoperative complications, objective hearing outcomes, and number of postoperative hospital and out of hospital visits.
CONCLUSIONS: A day-case approach to cochlear implant surgery does not result in a statistically significant reduction of health care-related costs compared to an inpatient approach and does not affect the surgical outcome (complications and objective hearing measurements), QoL, and postoperative course (number of postoperative hospital and out of hospital visits).
METHODS:

UNASSIGNED: To describe outcomes after bilateral cochlear implantation (CI) in a patient with a pathologic PTPN11 variant associated with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML). Additionally, to assess the utility of CI in this specific population based on our outcome and previous reports.
UNASSIGNED: Retrospective case report with literature review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
UNASSIGNED: A young boy with various multiorgan abnormalities, speech and language delay, and persistent hearing loss who was found to have a heterozygous PTPN11 gene mutation at age 2.
UNASSIGNED: Bilateral tympanostomy tube placement, diagnostic imaging, and eventual staged bilateral CI.
UNASSIGNED: Objective audiometric testing and developmental milestone attainment.
UNASSIGNED: Bilateral CI was successfully completed over a 2-month period. The patient illustrated significant improvement in objective audiologic measurement. However, he continues to sign as his main form of communication without significant speech progression.
UNASSIGNED: Early diagnostic and therapeutic intervention in patients with NS/NSML can help improve long-term audiologic and speech development. Given the heterogeneity of NS/NSML, a multidisciplinary approach is needed for optimal outcomes.

BACKGROUND: Wolfram-like syndrome (WFLS) is an autosomal dominant inherited disease characterized by a single heterozygous pathogenic variant in the WFS1 gene. Its clinical presentation is similar to autosomal recessive Wolfram syndrome.
METHODS: We reported a case of a 10-year-old boy and his family members who initially experienced hearing impairment (HI), followed by optic atrophy. Genetic testing revealed the presence of a WFS1 variant (chr4-6302385 exon8 NM_006005.3: c.2590G > A, p. Glu864Lys).
CONCLUSIONS: Wolfram-like syndrome, a rare neurodegenerative genetic disorder, manifested as deafness, optic atrophy, and diabetes mellitus. There hasn\'t been a definite treatment yet. Early identification of the variant in the WFS1 gene is beneficial for genetic counseling.

We present a patient who suffered a temporal bone fracture (TBF) encompassing the bony labyrinth. Sensorineural hearing loss was confirmed with an unfavorable prognosis for recovery. Thirteen years later, there is regression of the hearing loss.

OBJECTIVE: To report two new variants of ALMS1 gene and to discuss the audiological evolution and clinical phenotype in two pairs of siblings with Alström syndrome.
METHODS: This paper is a multi-disciplinary diagnostic evaluation, with genetic and audiological analysis that aims to report two new variants of the ALMS1 gene and to discuss the audiological evolution and clinical phenotype in a case series of patients with familial Alström syndrome. Therefore, we describe 4 cases presenting a complete audiometric profile of two pairs of unrelated siblings, to provide a better understanding of this very rare disease. Additionally, the present study identified two heterozygous mutations in the ALMS1 gene.
CONCLUSIONS: This Clinical Capsule Report highlights the importance of audiological monitoring throughout the development of patients with Alström syndrome. The two variants found were not previously reported in the literature, which expands the spectrum of ALMS1 variants in Alström syndrome.