Rare & Orphan Lab · DeCure for X

DeCure for Hearing loss, autosomal dominant 82

DeCure's autonomous Rare AI scientist is researching a drug-repurposing hypothesis for hearing loss, autosomal dominant 82 — screening already-approved drugs against its 1-gene Open Targets disease module to publish open-access research. Research is fast; the path to publication is funded in milestone stages.

Disease module1 genesLead labRare & Orphan
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Rare & OrphanDOID:0070603$DeCureRare

The disease map

Disease moduleHearing loss, autosomal dominant 82 maps to a 1-gene Open Targets module — the target space DeCure's AI scientist screens approved drugs against.
DeCure.ai methodSignature reversal (LINCS) plus network proximity (STRING) rank already-approved drugs likely to perturb this module — the same engine that produces DeCure.ai's repurposing hypotheses.
Repurposing thesisScreening approved medicines against this disease module, then publishing the evidence for the strongest candidate. Known pharmacology and human exposure data make the first question sharper — they do not establish safety or efficacy in a new indication.

Research record

01
ResearchComing soon
Candidate research + dossier — target rationale, drug-repurposing thesis and evidence pack.proof: Published dossier + on-chain hash
02
ValidationComing soon
In-vitro biological validation at a contract research org (CRO).proof: CRO contract + in-vitro report
03
Peer review & paperComing soon
Peer-reviewed paper published open-access (preprint + journal).proof: DOI + open-access link + on-chain hash

Current lead

No approved-drug candidate for hearing loss, autosomal dominant 82 is corroborated in the literature DeepSearch retrieved. Some conditions are managed with non-pharmacological care — a device, surgery or physical therapy — rather than a medicine; that may be the case here, or the literature we found may simply be too sparse yet to support a drug-repurposing angle.

What the evidence adds up to

The five abstracts provided contain no mention of any drug being tested or repurposed for hearing loss, autosomal dominant 82 or any other form of hearing loss. The 1978 symposium piece discusses general medical management and the otolaryngologist’s responsibility, but offers no specific pharmacological intervention. The 2013 study on cochlear implantation reports outcomes for 14 patients deafened by ototoxic drugs and 13 with sudden sensorineural hearing loss; post-operative BKB scores ranged from 33 to 100% (median 91%) in the ototoxic group and 16 to 100% (median 88%) in the sudden loss group, with no statistically significant difference between groups (P = 0.983). The authors note that outcomes may depend on the underlying pathology for which the ototoxic agents were prescribed.

The 2025 review on gene therapy for hereditary hearing loss describes three strategies—gene replacement, suppression, and editing—and summarises preclinical trials, but reports no completed human trials with efficacy data for any drug. The 1995 review on clinical methods in genetic hearing loss presents a pipeline from mutation identification to gene-based therapy, but states that each mutation is at a different stage in that pipeline and that gene-based therapy remains a future goal. The 1991 Spanish-language abstract (title translates to "Proclamations of the anticipation of death") comments on the complex genetic landscape of hearing loss and the limitations of whole-exome sequencing in achieving a 100% diagnostic rate, without reporting any therapeutic intervention. The 2025 expert consensus on surgical treatment for hereditary hearing loss focuses on surgical methods, efficacy evaluation, and postoperative rehabilitation, not on drug therapy.

No drug, repurposed or otherwise, is evaluated in any of these abstracts. What is missing for any potential drug-repurposing effort in autosomal dominant hearing loss 82 is a specific molecular target validated in human patients, a preclinical model that recapitulates the human mutation, funding for a clinical trial, and a trial design that accounts for the genetic heterogeneity and phenotypic diversity noted across these papers. Without those elements, no claim of efficacy can be made.

Evidence

Retrieved by DeepSearch across 234,678,978 indexed works and resolved on OpenAlex — ranked by citations, including the results that did not work.

The Laryngoscope · 1978 · 16 citations

Symposium on hearing loss ‐ The otolaryngologist's responsibility.: Medical management of hearing loss.

AbstractMillions of Americans suffer hearing loss resulting in immense social and economic consequences. Hearing loss is merely a symptom or sign and the evaluation and management of afflicted individuals requires a thorough knowledge of etiologic factors and understanding of the underlying pathophysiology. Hearing loss is either conductive, sensorineural, or mixed. It may be congenital or delayed in onset, genetic or progressive or stable. Specific diagnosis should be sought in all cases with the objective being reversal of the hearing loss. Often definitive therapy is lacking yet prevention of progression, when possible; recognition of associated disorders, when present; compensation for disability, when applicable; epidemiologic study; genetic and psycho-social counseling; and habilitation and rehabilitation may still be initiated. A vigorous approach to the patient with hearing loss should be championed by the otolaryngologist.

https://doi.org/10.1288/00005537-197806000-00007
Cochlear Implants International · 2013 · 14 citations

Cochlear implantation in patients deafened by ototoxic drugs

AbstractOBJECTIVE: To investigate the outcome of cochlear implantation (CI) in patients deafened by ototoxic drugs and to compare this, with the outcome of CI in sudden sensorineural hearing loss (SSNHL) with a similar duration of deafness. METHODS: The Manchester Auditory Implant Centre database was reviewed to identify patients who were implanted to rehabilitate profound sensorineural hearing loss resulting from treatment with ototoxic agents and patients with SSNHL group. A retrospective case note review of selected patients was carried out. Primary outcome measure was post-implantation Bamford-Kowal-Bench (BKB) score in quiet in both the groups. Secondary outcome measure was any significant complications following implantation. RESULTS: We identified 14 patients in the ototoxic group, which were matched with 13 patients in the SSNHL group. The post-operative BKB score in the ototoxic group ranged from 33 to 100% (median score 91%). One patient had bilateral CI. One patient required explantation following an infection. The post-operative BKB score in the SSNHL group ranged from 16 to 100% (median score 88%). One patient in this group could not be tested using this method as they did not have open set speech discrimination. Two patients in this group had bilateral CI. The data were analysed using Mann-Whitney U test. There was no statistically significant difference in the BKB scores in the two groups of patients (P value -0.983). CONCLUSION: Patients with profound hearing loss secondary to ototoxic agents can be rehabilitated successfully with CI. The outcomes may be variable and may be dependent on the underlying pathology for which the ototoxic agents were prescribed.

https://doi.org/10.1179/1754762812y.0000000020
Archives of Otolaryngology - Head and Neck Surgery · 1951 · 8 citations

IS HEARING LOSS DUE TO NUTRITIONAL DEFICIENCY? Further Studies on the Influence of Vitamin A in Certain Types of Impaired Hearing

AbstractIN A PRELIMINARY report,<sup>1</sup>a short time ago, I presented my experience with a new injectable vitamin A preparation in certain types of impaired hearing. Since the publication of this paper, Anderson, Zoller and Alexander<sup>2</sup>have conducted some interesting studies with the same preparation and concluded "that parenteral vitamin A therapy in massive doses is a valuable therapeutic agent in patients with deafness not amenable to the usual otolaryngologic methods of treatment." These studies have aroused considerable interest, not only in the newer therapy suggested but in some of the older theories which were advanced in an effort to show a relation between nutritional deficiency and hardness of hearing. This paper, therefore, has a twofold purpose, viz., (1) to clarify to some extent the possible role of vitamins in deafness and (2) to present results of more extended studies with anatola®<sup>3</sup>an improved injectable vitamin A product.

https://doi.org/10.1001/archotol.1951.03750050035003
Sensory Neuroscience · 2025 · 7 citations · open access

Update on Gene Therapy in the Treatment of Hereditary Hearing Loss

AbstractABSTRACT Gene therapy is a promising therapeutic approach for genetic disorders, involving genetic modification to repair or reconstruct faulty genetic material. It is particularly relevant to hereditary hearing loss (HHL), a common monogenic condition that can lead to congenital deafness. The recent approval of clinical trial results using gene therapy for HHL underscores the growing interest in this field. To further advance inner ear gene therapy and its application in genetic diseases, it is crucial to review the progress of gene therapy for HHL. This review focuses on the three major gene therapy strategies—gene replacement, gene suppression, and gene editing—highlighting their application across different monogenic disorders and successful preclinical trials in HHL. We summarize the primary gene therapy strategies used in recent years, discuss recent achievements in preclinical studies, and explore potential advancements in this field.

https://doi.org/10.1002/sen2.70004
Current Opinion in Otolaryngology & Head & Neck Surgery · 1995 · 2 citations

Modern clinical methods in genetic hearing loss

AbstractClinical methods in cases of genetic hearing loss have been influenced recently by both methodologic advances and specific findings made in the many research laboratories devoted to human genetic investigation. For the clinician, reports from these laboratories seem to suggest significant breakthroughs accompanied by allusions to clinical applicability. This review presents a rationale for the analysis of the many reports related to genetic hearing loss and their attending clinical impact. This rationale is based on a simplified view of the laboratory effort as a pipeline. A disease (mutation) passes through this pipeline on its way from family identification, through linkage analysis (chromosome localization), to gene sequencing and disease mechanism identification, and finally to gene-based therapy. Each stage of the investigation process has its own impact on clinical methods, and each mutation is in a particular spot in the pipeline at present. The pipeline itself is presented in this paper, along with the nature of the clinical impact of each level of laboratory findings.

https://doi.org/10.1097/00020840-199510000-00007
Advanced Science · 2025 · 1 citations · open access

Hearing Loss: From Basic to Clinical Science

AbstractHearing loss (HL) affects over 1.5 billion people globally, with genetic factors accounting for ≈50% of congenital cases. Therefore, HL has become a global health issue, driving extensive research from basic science to clinical applications. This Special Collection includes a total of 31 papers, among which 9 are review papers, 21 are research article papers, 1 is a perspective paper, that highlight the basic mechanisms and possible protection methods of HL, the application of gene therapy for treating HL, and the clinical study and application in HL.

https://doi.org/10.1002/advs.202521526
Revista de occidente · 1991 · 0 citations

Proclamas de la antelación de la muerte

AbstractOur findings confirm the complex genetic landscape of hearing loss and the limitations of WES in achieving a 100% diagnostic rate, especially in conditions characterized by genetic heterogeneity. These results contribute to our understanding of the genetic basis of hearing loss and emphasize the need for further research and comprehensive genetic analyses to elucidate the underlying causes of this condition.

https://doi.org/10.1186/s40246-024-00630-8
PubMed · 2025 · 0 citations

[Expert consensus on surgical treatment for hereditary hearing loss].

AbstractHereditary hearing loss, with its well-defined molecular etiology, is a typical disease suitable for applying the concept of individualized precision medicine to clinical practice. Given its genetic heterogeneity and phenotypic diversity, there are particularities for the surgical treatment of hereditary hearing loss. Based on the results of the molecular epidemiological survey of large samples of deafness, various surgical methods and postoperative follow-up data, this expert consensus formulates a detailed guidance plan for the surgical treatment, efficacy evaluation and postoperative rehabilitation of hereditary hearing loss.

https://doi.org/10.3760/cma.j.cn112137-20241230-02964

Disease module: DeepOracle (Open Targets). Structures: RDKit from PubChem SMILES. Literature: retrieved by DeepSearch across 234,678,978 indexed works (targeted per-candidate search), resolved on OpenAlex.

DeCure is a research and publication project, not medical advice and not a treatment. "DeCure for X" describes a research goal, not a claim that a cure exists. Backing a cure is a contribution to fund the research — it is not an investment, and confers no yield, royalty, equity or IP ownership. Papers are published open-access by the DeCure.ai DAO.