OBJECTIVE: To determine the cost-effectiveness or cost-utility of cochlear implants (CI) in adults with severe to profound bilateral hearing loss.
UNASSIGNED: PubMed (Medline), The Cochrane Library, Embase via Elsevier, EBSCOhost CINAHL, and Scopus.
METHODS: The study included adult participants with severe to profound bilateral neurosensory hearing loss. The analysis encompassed quality of life improvements, costs, cost-effectiveness, cost-utility, cost-benefit, quality-adjusted life year (QALY), incremental cost-effectiveness ratio (ICER), and incremental cost-utility ratio (ICUR). Systematic reviews, meta-analyses, case series, and prospective or retrospective cohort studies published in English between 2010 and 2023 were retrieved. Exclusion criteria included incomplete studies, abstracts, clinical cases, editorials, letters, studies involving pediatric populations, single-side deafness, methodology research, noneconomic aspects of CI, mixed child and adult data, and studies published before 2010. The risk of bias was assessed following the criteria outlined in Appendix I of the economic evaluation\'s quality assessment as per the NICE Guideline Development Method.
RESULTS: Ten articles met the criteria and were included in the qualitative synthesis. One study conducted a prospective cost-utility analyses, one carried out a cost-benefit analyses, one was a randomized controlled clinical trial focusing on cost-utility, and another was a clinical trial addressing cost-effectiveness. Six studies employed Markov models, and one study utilized uniquely the Monte Carlo method. None quantified the economic impact of improved hearing on cognitive function.
CONCLUSIONS: The heterogeneity of sources impacted data quality. Unilateral and sequential bilateral CI appeared to be cost-effective when compared with bilateral hearing aids or nontechnological support. When properly indicated, simultaneous bilateral CIs are cost-effective compared to no interventions and to unilateral cochlear implantation through differential discounting or variations from the base cases, especially with a life expectancy of 5-10 years or longer.

OBJECTIVE: To comprehensively examine the characteristics and prognosis of bilateral sudden sensorineural hearing loss (BSSHL) and its subtypes compared to unilateral sudden sensorineural hearing loss (USSHL).
METHODS: PubMed, Scopus, and CINAHL.
METHODS: Databases were searched from inception to December 5, 2023, for studies reporting patient characteristics and audiometric outcomes for BSSHL and its simultaneous (Si-BSSHL) and sequential (Se-BSSHL) subtypes. Meta-analysis of continuous measures, proportions (%), mean differences (Δ), and odds ratio (OR) were performed.
RESULTS: Eleven studies were included, consisting of 368 patients with BSSHL and 2,705 patients with USSHL. The pooled prevalence among all SSHL cases was 88.1% (95% CI: 81.2%-93.6%) for USSHL and 11.9% (95% CI: 6.4% to 18.8%) for BSSHL. PTA improvement following treatment with steroids was significantly worse in patients with BSSHL (Δ15.3 dB; 95% CI: 14.6 to 15.9; p < 0.0001) compared to patients with USSHL. There was no significant difference in post-treatment PTA improvement between the BSSHL subtypes. Patients with Si-BSSHL were significantly less likely to have an idiopathic etiology (OR: 0.4; 95% CI: 0.2 to 0.8; p = 0.01) and significantly more likely to have an autoimmune disease etiology (OR: 27.4; 95% CI: 2.2 to 336.1; p = 0.01), comorbid cardiovascular disease (OR: 2.3; 95% CI: 1.1 to 5.1; p = 0.03), and comorbid hypertension (OR: 2.5; 95% CI: 1.6 to 3.8; p < 0.0001) compared to patients with USSHL.
CONCLUSIONS: BSSHL is a considerably rarer form of SSHL with worse prognosis compared to USSHL. BSSHL, and Si-BSSHL in particular, has significantly greater associations with systemic pathologies compared to USSHL. Laryngoscope, 134:3883-3891, 2024.

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

OBJECTIVE: Bilateral, sudden sensorineural hearing loss (SSNHL) in the pediatric population is a rare phenomenon potentially detrimental to language acquisition and social development. This study comprehensively reviews and analyzes existing literature to determine any correlation or commonality in etiologies, presentations, and management of this condition.
METHODS: PubMed, Cochrane, Scopus, and Web of Science databases were systematically searched for articles related to pediatric SSNHL from 1970 to 2021. Case series, case reports, and cohort studies were included. Data on patient demographics, etiology, diagnostic testing, management, and hearing recovery were collected.
RESULTS: Excluding duplicates, 553 unique titles were identified by established search criteria, of which 342 titles were relevant to pediatric sudden hearing loss. Forty-six papers reported cases of bilateral SSNHL, totaling 145 individual cases. Not included in the analysis were 45 cases documented as non-organic hearing loss. The average age of the total 145 included patients was 8.5 years and 51 were male. Reported etiologies included cytomegalovirus (n = 3), meningitis (n = 13), mumps (n = 5), ototoxin exposure (n = 13), and enlarged vestibular aqueduct (n = 9). Tinnitus (n = 30) was the most reported concurrent symptom, followed by vertigo (n = 21). Systemic steroid therapy was the most common treatment and, when follow up was reported, most patients (51.2%) had complete or partial recovery of hearing.
CONCLUSIONS: This is a comprehensive review of pediatric bilateral SSNHL. Though often idiopathic, etiologies also include infectious, structural, and autoimmune. Treatment largely consists of systemic steroid therapy, with variables rates of recovery. Further studies on intratympanic administration of steroids may guide future treatment.

To perform a systematic review of sensorineural hearing loss (SNHL) in hematologic malignancy; to describe an illustrative case of urgent cochlear implantation for bilateral profound SNHL and vestibular hypofunction in hyperviscosity syndrome; to suggest an approach to management of hyperviscosity syndrome-associated deafness with cochlear implantation.
Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, systematic search of PubMed and Embase databases was undertaken for articles detailing clinical information about SNHL caused directly by hematologic malignancies.
A total of 37 studies from 1989 to 2020 were qualitatively reviewed, the majority of which were case studies or case series. Causes of hearing loss in hematologic malignancy were found to include hyperviscosity syndrome, labyrinthine hemorrhage, infiltration, and infection. Patients with profound SNHL in hematologic malignancies from hyperviscosity syndrome may be candidates for cochlear implantation, and are also at increased risk for cochlear ossification. We review previous cases for their diagnostic approach, treatment paradigm, and outcomes data, and propose an approach to management.
Bilateral sudden profound SNHL and vestibular hypofunction is a presenting symptom of hyperviscosity syndrome in hematologic malignancy. Although this entity is rare and previous reports have suggested improvement in hearing with oncologic treatment, cases with profound hearing loss are unlikely to recover serviceable hearing. We advocate for early magnetic resonance imaging with attention to fluid signal in the inner ear and serial audiometric follow-up to guide clinical decisions. We advise early consideration for cochlear implantation.

Townes-Brocks syndrome (TBS) is a rare autosomal dominant syndrome, resulting from heterozygous variant in SALL1 gene and initially characterized by the triad of anorectal, thumb, and ear malformations. Essentially described in children, adult case reports are uncommon. Renal involvement has already been reported in adults and children but poorly described. Structural abnormalities such as hypodysplasia, unilateral renal agenesis or multicystic kidneys have been described, as well as functional impairment (with or without structural abnormalities) that may progress to end-stage renal disease (ESRD). We report two adult cases (mother and daughter) which exhibited kidney hypoplasia (focal and segmental glomerulosclerosis for the mother) and ESRD. The mother had unilateral polydactyly. TBS was suggested after physical examination. TBS diagnosis was confirmed by identification of a SALL1 variant. We conducted a literature review to evaluate the renal anomalies in TBS cases diagnosed in adulthood. Among 44 adult cases of TBS with genetic confirmation (including our two cases), 10 had kidney disease. The circumstances of renal failure diagnosis were incidental findings (2/5), gout (2/5), or repeated episodes of pyelonephritis (1/5). The median age of kidney disease diagnosis was 30 years old and of renal transplant 49 years old. The most frequent renal malformation was bilateral kidney hypoplasia. TBS is probably underestimated in adulthood and this report highlights that less obvious elements of morphology such as dysplasic ears can facilitate the diagnosis of TBS. As long-term prognosis of renal involvement in TBS patients remains largely unknown, a regular evaluation is required throughout life for patients.

We have investigated the main genetic causes for non-syndromic hearing impairment (NSHI) in the hearing impairment individuals from the North-Eastern Romania and proposed a cost-effective diagnosis protocol.
MLPA followed by Sanger Sequencing were used for all 291 patients included in this study.
MLPA revealed abnormal results in 141 cases (48.45%): 57 (40.5%) were c.35delG homozygous, 26 (18.44%) were c.35delG heterozygous, 14 (9.93%) were compound heterozygous and 16 (11.35%) had other types of variants. The entire coding region of GJB2 was sequenced and out of 150 patients with normal results at MLPA, 29.33% had abnormal results: variants in heterozygous state: c.71G>A (28%), c.457G>A (20%), c.269T>C (12%), c.109G>A (12%), c.100A>T (12%), c.551G>C (8%). Out of 26 patients with c.35delG in heterozygous state, 38.46% were in fact compound heterozygous.
We identified two variants: c.109G>A and c.100A>T that have not been reported in any study from Romania. MLPA is an inexpensive, rapid and reliable technique that could be a cost-effective diagnosis method, useful for patients with hearing impairment. It can be adaptable for the mutation spectrum in every population and followed by Sanger sequencing can provide a genetic diagnosis for patients with different degrees of hearing impairment.

Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine.
To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL.
This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019.
A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting.
In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement).
These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

OBJECTIVE: The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature.
METHODS: A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases.
RESULTS: A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL.
CONCLUSIONS: Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling.

Objective: To investigate the variance in reported prevalence rates of permanent neonatal hearing impairment (HI) worldwide.Design: A systematic review and meta-analysis was performed on reported prevalence rates of sensorineural and permanent conductive or mixed HI worse than 40 dB in neonates, detected as a result of a screening programme or audiometric study.Study sample: For meta-analysis, 35 articles were selected, 25 from high-income countries and 10 from middle-income countries according to the world bank classification system.Results: The prevalence rate of permanent uni- and bilateral HI worse than 40 dB in neonates varied from 1 to 6 per 1000, the overall prevalence was 2.21 per 1000 [1.71, 2.8]. In NICU populations the prevalence rate was higher with a larger fraction of bilateral cases. Although not significant, prevalence rates were slightly higher in Asia compared to Europe and the number of infants lost to follow-up appeared higher in countries with lower gross national income.Conclusion: Substantial variations exist in prevalence rates of neonatal permanent HI across countries and regions. There is a strong need for more data from low-income countries to identify demographic factors that account for this variability in reported prevalence rates. Reporting these data in a uniform way is advocated.