Browsing by Subject "Deafness"

Now showing 1 - 10 of 29
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    An analysis of hearing aid fittings in adults using cochlear implants and contralateral hearing aids
    (Wiley, 2010-12) Harris, Michael S.; Hay-McCutcheon, Marcia; Otolaryngology -- Head and Neck Surgery, School of Medicine
    OBJECTIVES/HYPOTHESIS: The objective of this study was to assess the appropriateness of hearing aid fittings within a sample of adult cochlear implant recipients who use a hearing aid in the contralateral ear (i.e., bimodal stimulation). METHODS: The hearing aid gain was measured using real ear testing for 14 postlingually deaf English-speaking adults who use a cochlear implant in the contralateral ear. Unaided and aided audiometric testing assessed the degree of functional gain derived from hearing aid use. RESULTS: On average, the target to actual output level difference was within 10 dB only at frequencies of 750 Hz and 1,000 Hz. Only 1 of the 14 study participants had a hearing aid for which the majority of the tested frequencies were within 10 dB of the target gain. In addition, a greater amount of functional gain (i.e., the increase in unaided behavioral thresholds after amplification) was provided for lower frequencies than higher frequencies. CONCLUSIONS: Hearing aid settings in our sample were suboptimal and may be regarded as a contributing factor to the variability in bimodal benefit. Refining hearing aid fitting strategies tailored to the needs of the concurrent cochlear implant and hearing aid user is recommended.
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    Consensus clinical management guidelines for Alström syndrome
    (BMC, 2020-09-21) Tahani, Natascia; Maffei, Pietro; Dollfus, Hélène; Paisey, Richard; Valverde, Diana; Milan, Gabriella; Han, Joan C.; Favaretto, Francesca; Madathil, Shyam C.; Dawson, Charlotte; Armstrong, Matthew J.; Warfield, Adrian T.; Düzenli, Selma; Francomano, Clair A.; Gunay-Aygun, Meral; Dassie, Francesca; Marion, Vincent; Valenti, Marina; Leeson-Beevers, Kerry; Chivers, Ann; Steeds, Richard; Barrett, Timothy; Geberhiwot, Tarekegn; Medical and Molecular Genetics, School of Medicine
    Alström Syndrome (ALMS) is an ultra-rare multisystem genetic disorder caused by autosomal recessive variants in the ALMS1 gene, which is located on chromosome 2p13. ALMS is a multisystem, progressive disease characterised by visual disturbance, hearing impairment, cardiomyopathy, childhood obesity, extreme insulin resistance, accelerated non-alcoholic fatty liver disease (NAFLD), renal dysfunction, respiratory disease, endocrine and urologic disorders. Clinical symptoms first appear in infancy with great variability in age of onset and severity. ALMS has an estimated incidence of 1 case per 1,000,000 live births and ethnically or geographically isolated populations have a higher-than-average frequency. The rarity and complexity of the syndrome and the lack of expertise can lead to delayed diagnosis, misdiagnosis and inadequate care. Multidisciplinary and multiprofessional teams of experts are essential for the management of patients with ALMS, as early diagnosis and intervention can slow the progression of multi-organ dysfunctions and improve patient quality of life. These guidelines are intended to define standard of care for patients suspected or diagnosed with ALMS of any age. All information contained in this document has originated from a systematic review of the literature and the experiences of the authors in their care of patients with ALMS. The Appraisal of Guidelines for Research & Evaluation (AGREE II) system was adopted for the development of the guidelines and for defining the related levels of evidence and strengths of recommendations. These guidelines are addressed to: a) specialist centres, other hospital-based medical teams and staffs involved with the care of ALMS patients, b) family physicians and other primary caregivers and c) patients and their families.
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    Deafness-in-a-dish: modeling hereditary deafness with inner ear organoids
    (Springer, 2022) Romano, Daniel R.; Hashino, Eri; Nelson, Rick F.; Otolaryngology -- Head and Neck Surgery, School of Medicine
    Sensorineural hearing loss (SNHL) is a major cause of functional disability in both the developed and developing world. While hearing aids and cochlear implants provide significant benefit to many with SNHL, neither targets the cellular and molecular dysfunction that ultimately underlies SNHL. The successful development of more targeted approaches, such as growth factor, stem cell, and gene therapies, will require a yet deeper understanding of the underlying molecular mechanisms of human hearing and deafness. Unfortunately, the human inner ear cannot be biopsied without causing significant, irreversible damage to the hearing or balance organ. Thus, much of our current understanding of the cellular and molecular biology of human deafness, and of the human auditory system more broadly, has been inferred from observational and experimental studies in animal models, each of which has its own advantages and limitations. In 2013, researchers described a protocol for the generation of inner ear organoids from pluripotent stem cells (PSCs), which could serve as scalable, high-fidelity alternatives to animal models. Here, we discuss the advantages and limitations of conventional models of the human auditory system, describe the generation and characteristics of PSC-derived inner ear organoids, and discuss several strategies and recent attempts to model hereditary deafness in vitro. Finally, we suggest and discuss several focus areas for the further, intensive characterization of inner ear organoids and discuss the translational applications of these novel models of the human inner ear.
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    Defective Tmprss3-Associated Hair Cell Degeneration in Inner Ear Organoids
    (Elsevier, 2019-07-09) Tang, Pei-Ciao; Alex, Alpha L.; Nie, Jing; Lee, Jiyoon; Roth, Adam A.; Booth, Kevin T.; Koehler, Karl R.; Hashino, Eri; Nelson, Rick F.; Otolaryngology, IU School of Medicine
    Mutations in the gene encoding the type II transmembrane protease 3 (TMPRSS3) cause human hearing loss, although the underlying mechanisms that result in TMPRSS3-related hearing loss are still unclear. We combined the use of stem cell-derived inner ear organoids with single-cell RNA sequencing to investigate the role of TMPRSS3. Defective Tmprss3 leads to hair cell apoptosis without altering the development of hair cells and the formation of the mechanotransduction apparatus. Prior to degeneration, Tmprss3-KO hair cells demonstrate reduced numbers of BK channels and lower expressions of genes encoding calcium ion-binding proteins, suggesting a disruption in intracellular homeostasis. A proteolytically active TMPRSS3 was detected on cell membranes in addition to ER of cells in inner ear organoids. Our in vitro model recapitulated salient features of genetically associated inner ear abnormalities and will serve as a powerful tool for studying inner ear disorders.
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    Differential At-Risk Pediatric Outcomes of Parental Sensitivity Based on Hearing Status
    (American Speech-Language-Hearing Association, 2021) Jamsek, Izabela A.; Holt, Rachael Frush; Kronenberger, William G.; Pisoni, David B.; Psychiatry, School of Medicine
    Purpose: The aim of this study was to investigate the role of parental sensitivity in language and neurocognitive outcomes in children who are deaf and/or hard of hearing (DHH). Method: Sixty-two parent–child dyads of children with normal hearing (NH) and 64 of children who are DHH (3–8 years) completed parent and child measures of inhibitory control/executive functioning and child measures of sentence comprehension and vocabulary. The dyads also participated in a video-recorded, free-play interaction that was coded for parental sensitivity. Results: There was no evidence of associations between parental sensitivity and inhibitory control or receptive language in children with NH. In contrast, parental sensitivity was related to children's inhibitory control and all language measures in children who are DHH. Moreover, inhibitory control significantly mediated the association between parental sensitivity and child language on the Clinical Evaluation of Language Fundamentals–Fifth Edition Following Directions subscale (6–8 years)/Clinical Evaluation of Language Fundamentals Preschool–Second Edition Concepts and Following Directions subscale (3–5 years). Follow-up analyses comparing subgroups of children who used hearing aids (n = 29) or cochlear implants (CIs; n = 35) revealed similar correlational trends, with the exception that parental sensitivity showed little relation to inhibitory control in the group of CI users. Conclusions: Parental sensitivity is associated with at-risk language outcomes and disturbances in inhibitory control in young children who are DHH. Compared to children with NH, children who are DHH may be more sensitive to parental behaviors and their effects on emerging inhibitory control and spoken language. Specifically, inhibitory control, when scaffolded by positive parental behaviors, may be critically important for robust language development in children who are DHH.
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    Exceptional Speech Recognition Outcomes After Cochlear Implantation: Lessons From Two Case Studies
    (American Speech-Language-Hearing Association, 2022) Herbert, Carolyn J.; Pisoni, David B.; Kronenberger, William G.; Nelson, Rick F.; Otolaryngology -- Head and Neck Surgery, School of Medicine
    Purpose: Individual differences and variability in outcomes following cochlear implantation (CI) in patients with hearing loss remain significant unresolved clinical problems. Case reports of specific individuals allow for detailed examination of the information processing mechanisms underlying variability in outcomes. Two adults who displayed exceptionally good postoperative CI outcomes shortly after activation were administered a novel battery of auditory, speech recognition, and neurocognitive processing tests. Method: A case study of two adult CI recipients with postlingually acquired hearing loss who displayed excellent postoperative speech recognition scores within 3 months of initial activation. Preoperative City University of New York sentence testing and a postoperative battery of sensitive speech recognition tests were combined with auditory and visual neurocognitive information processing tests to uncover their strengths, weaknesses, and milestones. Results: Preactivation CUNY auditory-only (A) scores were < 5% correct while the auditory + visual (A + V) scores were > 74%. Acoustically with their CIs, both participants' scores on speech recognition, environmental sound identification and speech in noise tests exceeded average CI users scores by 1-2 standard deviations. On nonacoustic visual measures of language and neurocognitive functioning, both participants achieved above average scores compared with normal hearing adults in vocabulary knowledge, rapid phonological coding of visually presented words and nonwords, verbal working memory, and executive functioning. Conclusions: Measures of multisensory (A + V) speech recognition and visual neurocognitive functioning were associated with excellent speech recognition outcomes in two postlingual adult CI recipients. These neurocognitive information processing domains may underlie the exceptional speech recognition performance of these two patients and offer new directions for research explaining variability in postimplant outcomes. Results further suggest that current clinical outcome measures should be expanded beyond the conventional speech recognition measures to include more sensitive robust tests of speech recognition as well as neurocognitive measures of working memory, vocabulary, lexical access, and executive functioning.
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    Family Environmental Dynamics Differentially Influence Spoken Language Development in Children With and Without Hearing Loss
    (American Speech-Language-Hearing Association, 2022) Holt, Rachael Frush; Kronenberger, William G.; Pisoni, David B.; Psychiatry, School of Medicine
    Purpose: The aim of this study was to evaluate whether families of children with sensorineural hearing loss (SNHL) are organized similarly to those of typically developing, typically hearing (TH) children and whether the dimensions of family dynamics and environment are related to spoken language development similarly in children with and without SNHL. Method: Primary caregivers of children with SNHL (n = 63) or TH (n = 65) completed the Family Environment Scale-Fourth Edition (FES-4) to assess multiple dimensions of family environment. Children's receptive vocabulary was assessed with the Peabody Picture Vocabulary Test-Fourth Edition, and their receptive language was assessed by an age-appropriate version of the Concepts and Following Directions subtest of the Clinical Evaluation of Language Fundamentals and the Sentence Comprehension subscale of the Comprehensive Assessment of Spoken Language-Second Edition. Principal component analysis was used to examine the dimensional structure of the family environment. Results: Three higher order components were derived from FES-4 subscales for both families of children with SNHL and with TH: Supportive, Controlling, and Conflicted. However, the composition of the factors themselves differed between the two groups. For the TH group, most family environment measures on the FES-4 were not associated with language outcomes. In contrast, for children with SNHL, families who were more supportive, less controlling, and less conflicted had children with better language skills. Conclusions: Three well-accepted dimensions of family dynamics and functioning apply to families of children with SNHL, but their composition differs from those of families with TH children. Family environmental dynamics were much more strongly associated with language outcomes in children with SNHL than in their TH peers. The spoken language development of children with SNHL, in particular, is better in families that provide high levels of support for each other and, in particular, low levels of control, disorganization, and conflict, reflecting the fragile nature of their spoken language development.
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    Family-Level Executive Functioning and At-Risk Pediatric Hearing Loss Outcomes
    (American Speech-Language-Hearing Association, 2021) Blank, Andrew; Frush Holt, Rachael; Pisoni, David B.; Kronenberger, William G.; Otolaryngology -- Head and Neck Surgery, School of Medicine
    Purpose: Using a new measure of family-level executive functioning (EF; the Family Characteristics Scale [FCS]), we investigated associations between family-level EF, spoken language, and neurocognitive skills in children with hearing loss (HL), compared to children with normal hearing. Method: Parents of children with HL (n = 61) or children with normal hearing (n = 65) completed the FCS-Parent, and clinicians evaluated families using the FCS-Examiner. Children completed an age-appropriate version of the Concepts and Following Directions subtest of the Clinical Evaluation of Language Fundamentals and the Peabody Picture Vocabulary Test–Fourth Edition. Child EF was assessed via the parent report Behavior Rating Inventory of Executive Function. Results: Two higher order components were derived from FCS subscales: Family Inhibition and Family Organization. For both samples, Family Inhibition was positively associated with child inhibition, child shifting, and child language comprehension skills. Family Organization was differentially associated with child inhibition, working memory, and planning/organization skills across the samples. Additionally, Family Inhibition was associated with child planning and organization skills for children with HL. Conclusions: Results support the FCS as a measure of family-level EF. Family-level inhibition related to better child inhibition, flexibility/shifting, and language comprehension across both samples and to better planning and organization skills in children with HL. As children with HL experienced greater difficulties in EF, families demonstrated greater organization, possibly as a compensatory measure. Results suggest that inhibition and organization at a family level may be important targets for the development of novel interventions to promote EF and language outcomes for children with HL.
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    Functional Hearing Quality in Prelingually Deaf School-Age Children and Adolescents with Cochlear Implants
    (Taylor & Francis, 2021) Kronenberger, William G.; Bozell, Hannah; Henning, Shirley C.; Montgomery, Caitlin J.; Ditmars, Allison M.; Pisoni, David B.; Psychiatry, School of Medicine
    Objective: This study investigated differences in functional hearing quality between youth with cochlear implants (CIs) and normal hearing (NH) peers, as well as associations between functional hearing quality and audiological measures, speech perception, language and executive functioning (EF). Design: Youth with CIs and NH peers completed measures of audiological functioning, speech perception, language and EF. Parents completed the Quality of Hearing Scale (QHS), a questionnaire measure of functional hearing quality. Study sample: Participants were 43 prelingually-deaf, early-implanted, long-term CI users and 43 NH peers aged 7-17 years. Results: Compared to NH peers, youth with CIs showed poorer functional hearing quality on the QHS Speech, Localization, and Sounds subscales and more hearing effort on the QHS Effort subscale. QHS scores did not correlate significantly with audiological/hearing history measures but were significantly correlated with most speech perception, language and EF scores in the CI sample. In the NH sample, QHS scores were uncorrelated with speech perception and language and were inconsistently correlated with EF. Conclusions: The QHS is a valid measure of functional hearing quality that is distinct from office-based audiometric or hearing history measures. Functional hearing outcomes are associated with speech-language and EF outcomes in CI users.