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Inner Ear Conductive Hearing Loss: Current Studies and Controversies

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A special issue of Audiology Research (ISSN 2039-4349).

Deadline for manuscript submissions: 30 May 2025 | Viewed by 18782

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Dr. Andrea Castellucci

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ENT Unit, Department of Surgery, Azienda USL—IRCCS di Reggio Emilia, Reggio Emilia, Italy
Interests: neurotology; inner ear; vestibular disease; BPPV; Meniere’s disease; vestibular migraine; acute vestibular loss; canal dehiscence; sudden hearing loss; video-HIT; VEMPs
Special Issues, Collections and Topics in MDPI journals

Dr. Eduardo Martin Sanz

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1. Department of Otolaryngology, University Hospital of Getafe, Carretera Toledo km 12,500, 28905 Madrid, Spain
2. Department of Medicine, School of Biomedical Sciences and Health, European University of Madrid, C. Tajo, s/n, 28670 Madrid, Spain
Interests: vestibular function; meniere disease; vestibular compensation; vestibular restoration; vertigo; tinnitus; benign paroxysmal positional vertigo; vestibular neuritis
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Soumit Dasgupta

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Guest Editor

Department of Paediatric Audiology and Audiovestibular Medicine, Alder Hey Children’s, University of Liverpool, Liverpool, UK
Interests: paediatric balance; ototoxicity; genetics of hearing loss and head injuries

Prof. Dr. Issam Saliba

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Department of Otolaryngology-Head and Neck Surgery, Montreal University Hospital Center (CHUM), Montreal University, Montreal, QC, Canada
Interests: neurotology and skull base surgery; ménière’s disease; superior canal dehiscence syndrome; tympanoplasty; vestibular migraine; electrocochleography; vestibular disease

Special Issue Information

Dear Colleagues,

Conductive hearing loss (CHL) without middle ear abnormalities had generated an otological controversy and mystery for many years, often leading to uneventful surgical procedures. In recent years, it has been adequately evidenced as to how third window pathologies (TWP) could account for the so-called “pseudo-CHL” due to a reduction of bone-conducted threshold and a dissipation of air-conducted sounds for low frequencies in the pure tone audiometry given a low-impedance pathway for the cochlear travelling wave in the labyrinthine partition responsible for the appearance of an air bone gap (ABG). Nevertheless, besides the classical TWP due to a bony labyrinthine structural abnormality (i.e., canal dehiscence), several inner ear disorders affecting both adults and children have shown a similar CHL through different pathomechanisms. For example, early-stage Meniere’s Disease and Intralabyrinthine Schwannoma may result in CHL or mixed HL by interfering with the cochlear traveling wave and a perilymphatic fistula might lead to low-frequency ABG through a similar mechanism to TWP. Additionally, congenital or acquired round window pathologies and other labyrinthine disorders resulting in an abnormal physical or functional communication between inner ear and cerebrospinal fluid might result in similar ABG in audiometry. Examples include dilated vestibular aqueducts, dilated internal auditory meati and X-linked gusher syndrome. Laser-doppler vibrometry, wideband tympanometry and other tests exploring vestibular function (such as the video-head impulse test and vestibular-evoked myogenic potential tests) have led to a robust diagnosis of a third window due to any reason and have gained popularity in the differential diagnosis in CHL with normal middle ear function. It also needs to be borne in mind that TWP in children may present with different and variable phenotypes as compared to adults. The aim of this Special Issue is to provide new insights in this topic, facing possible clinical presentation, instrumental diagnosis and treatments strategies for inner ear CHL and shading light on controversies on underlying pathomechanisms. Research manuscripts and review on this topic are welcome.

Dr. Andrea Castellucci
Dr. Eduardo Martin Sanz
Prof. Dr. Soumit Dasgupta
Prof. Dr. Issam Saliba
Guest Editors

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Keywords

conductive hearing loss
round window
third window syndromes
enlarged vestibular aqueduct
canal dehiscence
perilymphatic fistula
meniere’s disease
intralabyrinthine schwannoma
wideband tympanometry
vestibular-evoked myogenic potentials

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Published Papers (10 papers)

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Research

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8 pages, 1429 KiB

Open AccessArticle

Air–Bone Gap in Meniere’s Disease: A Case Series and Literature Review

by Arun Pajaniappane, Nilesh Parekh and Anita Wong

Audiol. Res. 2024, 14(6), 983-990; https://doi.org/10.3390/audiolres14060081 - 8 Nov 2024

Viewed by 749

Abstract

Background/Objectives: An air–bone gap (ABG) on audiometry is usually secondary to a conductive hearing loss. However, persistent and repeatable ABGs on audiometry in the absence of external or middle ear pathology is thought to arise from inner ear disorders including Meniere’s Disease (MD). In this paper, we aim to showcase this interesting finding occurring in MD with an associated literature review. Methods: Using retrospective review and analysis of case notes, we describe eight cases of persistent ABG on audiometry in MD. All other causes for the ABG were explored and excluded with the aid of objective audiological testing. Results: ABG can occur in a small sub-set of the MD population. In our case series, the ABG was typically found to affect the low frequencies. Higher frequencies appear to be spared. However, a detailed history, examination and battery of objective tests are required to ensure that all other causes of the ABG are reliably considered and excluded prior to attributing it to the inner ear. The finding of an ABG in MD may be dependent on the stage of the disease. Further research is required to determine the underlying cause of the ABG and its potential applications to help guide treatment. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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15 pages, 2510 KiB

Open AccessArticle

Suspicion and Treatment of Perilymphatic Fistula: A Prospective Clinical Study

by Issam Saliba, Naif Bawazeer and Sarah Belhassen

Audiol. Res. 2024, 14(1), 62-76; https://doi.org/10.3390/audiolres14010006 - 8 Jan 2024

Viewed by 1956

Abstract

Background: Since the discovery of the perilymphatic fistula (PLF), the diagnosis and treatment remain controversial. If successfully recognized, the PLF is surgically repairable with an obliteration of the fistula site. Successful treatment has a major impact on patient’s quality of life with an improvement in their audiological and vestibular symptoms. Objective: To prospectively investigate patients’ clinical and audiological evolution with PLF suspicion after middle ear exploration and obliteration of the round and oval window. Study Design: Prospective comparative study. Setting: Tertiary care center. Methods: Patients were divided into two groups: Group I consisted of patients where no PLF had been identified intraoperatively at the oval and/or at the round window, and Group II consisted of patients where a fistula had been visualized. Patient assessment was a combination of past medical history, the presence of any risk factors, cochlear and vestibular symptoms, a physical examination, temporal bone imaging, audiograms, and a videonystagmogram (VNG). Results: A total of 98 patients were divided into two groups: 62 in Group I and 36 in Group II. A statistically significant difference regarding gender was observed in Group II (83.3% of males vs. 16.7% of females, p = 0.008). A total of 14 cases (4 and 10 in Groups I and II, respectively) were operated for a recurrent PLF. Fat graft material was used in the majority of their previous surgery; however, no difference was found when comparing fat to other materials. In addition, no statistically significant difference was noted between Groups I and II concerning predisposing factors, imaging, VNG, symptom evolution, or a physical exam before the surgery and at 12 months post-operative. However, both groups showed statistically significant hearing and vestibular improvement. On the other hand, the air conduction (AC) and bone conduction (BC) at each frequency were not statistically different between the two groups before surgery but showed statistically significant improvement at 12 months post-operatively, especially for the BC at the frequencies 250 (p = 0.02), 500 (p = 0.0008), and 1000 Hz (p = 0.04). Conclusions: Whenever you suspect a perilymphatic fistula, do not hesitate to explore middle ear and do window obliterations using a tragal perichondrium material. Our data showed that cochlear and vestibular symptoms improved whether a fistula had been identified or not. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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12 pages, 1827 KiB

Open AccessArticle

Cavitating Lesions around the Cochlea Can Affect Audiometric Threshold and Clinical Practice

by Giulia Zambonini, Sara Ghiselli, Giuseppe Di Trapani, Daria Salsi and Domenico Cuda

Audiol. Res. 2023, 13(5), 821-832; https://doi.org/10.3390/audiolres13050072 - 20 Oct 2023

Viewed by 1365

Abstract

There are several pathologies that can change the anatomy of the otic capsule and that can distort the bone density of the bony structures of the inner ear, but otosclerosis is one of the most frequent. Similar behavior has been shown in patients affected by osteogenesis imperfecta (OI), a genetic disorder due to a mutation in the genes coding for type I (pro) collagen. In particular, we note that otosclerosis and OI can lead to bone resorption creating pericochlear cavitations in contact with the internal auditory canal (IAC). In this regard, we have collected five cases presenting this characteristic; their audiological data and clinical history were analyzed. This feature can be defined as a potential cause of a third-window effect, because it causes an energy loss during the transmission of sound waves from the oval window (OW) away from the basilar membrane. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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15 pages, 2083 KiB

Open AccessArticle

X-Linked Gusher Disease DFNX2 in Children, a Rare Inner Ear Dysplasia with Mixed Hearing and Vestibular Loss

by Soumit Dasgupta, James Hong, Rhyanna Morris, Javed Iqbal, Amy Lennox-Bowley and Jeyasakthy Saniasiaya

Audiol. Res. 2023, 13(4), 600-614; https://doi.org/10.3390/audiolres13040052 - 4 Aug 2023

Cited by 4 | Viewed by 2997

Abstract

Conductive hearing losses are typically present in disorders of the external/middle ear. However, there is a rare group of inner ear conditions called third windows that can also generate a conductive hearing loss. This is due to an abnormal connection between the middle and the inner ear or between the inner ear and the cranial cavity. X-linked gusher disorder is an extremely rare congenital inner ear dysplastic syndrome with an abnormal connection due to a characteristic incomplete cochlear partition type III and an incomplete internal auditory meatus fundus. The disorder is inherited in an X-linked fashion due to the mutation of the POU3F4 gene. We present two siblings diagnosed with the condition and their long-term follow-ups. They both presented audiovestibular symptoms and showed progressive mixed losses and bilateral vestibular weakness. They were treated with cochlear implant, digital amplification and with vestibular rehabilitation. Significant others around them were involved in their journey with the medical team, and in both, a very favourable outcome was achieved. This is the first time that we have reported evolving audiovestibular function with vestibular quantification in X-linked gusher disorder and emphasize on the multidisciplinary holistic approach to manage these children effectively. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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12 pages, 2867 KiB

Open AccessArticle

Association between Third Mobile Window Disorders and Symptom Reduction Using a Noise Cancelling Device: Inverse Tullio Phenomena

by Debby Feinberg, Mark Rosner and Gerard Gianoli

Audiol. Res. 2023, 13(4), 516-527; https://doi.org/10.3390/audiolres13040046 - 17 Jul 2023

Viewed by 1567

Abstract

Identifying a vestibular source of pathology in patients complaining of post-traumatic brain injury (TBI) dizziness can be difficult. We describe a possible new method utilizing a reduction in post-TBI symptoms (including dizziness) with the use of a noise cancellation device (NCD). This retrospective case series included patients with TBI and dizziness presenting to a binocular vision specialty clinic, who were diagnosed with a vertical heterophoria (VH). If they did not respond adequately to microprism lenses and/or if they experienced hyperacusis, they were evaluated with an NCD. If there was marked reduction in TBI symptoms (including dizziness), the patients were referred to a neuro-otologist for vestibular diagnostic evaluation and treatment. Fourteen patients were identified and found to have abnormalities on vestibular testing consistent with third mobile window disorder (TMWD). All were treated with a 6-week medical protocol (diuretics, no straining, low sodium/no caffeine diet). Five responded positively, requiring no further treatment. Nine required surgical intervention and responded positively. In conclusion, in 14 patients with post-concussive dizziness and VH, a positive response to NCD was associated with abnormal vestibular testing, a diagnosis of TMWD, and symptom reduction/resolution with a medical or surgical approach. The removal of sound resulting in reduction or resolution of vestibular symptoms represents an inverse Tullio phenomenon. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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10 pages, 845 KiB

Open AccessArticle

Hearing Outcomes Following Endolymphatic Duct Blockage Surgery and Factors Associated with Improved Audition at Two Years Follow-Up

by Issam Saliba and Marc-Henri Asmar

Audiol. Res. 2023, 13(3), 431-440; https://doi.org/10.3390/audiolres13030038 - 2 Jun 2023

Cited by 1 | Viewed by 2032

Abstract

Objective: To evaluate hearing outcomes at 2 years post endolymphatic duct blockage (EDB) surgery, with an analysis of factors that may predict hearing improvement. Study Design: Retrospective comparative study. Setting: Tertiary care center. Subjects: Definite Ménière’s Disease (MD) patients undergoing EDB for refractory disease. Methods: Chart review was conducted to assign cases to one of the three hearing outcome groups (deteriorated, stable, and improved). All cases that met our inclusion criteria were selected. Preoperative data collected were audiograms, bithermal caloric tests, preoperative vertigo episodes, history of previous ear surgery for Ménière, intratympanic steroid injections (ITS) and intraoperative endolymphatic sac (ELS) tear or opening. Postoperative data collected at 24 months were audiograms, vertigo episodes and bithermal caloric testing. Results: Preoperative vertigo episodes, caloric paresis and history of surgery, ITS injections or ELS integrity, as well as postoperative vertigo class distribution and caloric paresis changes were not different between our groups. Preoperative word recognition score (WRS) was lowest in the improved hearing group (p = 0.032). The persistence of tinnitus at 2 years postoperatively was associated with deteriorated hearing (p = 0.033). Conclusions: There are no strong predictors of hearing improvement on presentation pre-EDB, but low preoperative WRS may be the best estimator available. Therefore, ablative interventions should be considered very carefully in patients presenting with low WRS, as they may benefit more from EDB; there is a fair chance of a good hearing outcome with EDB surgery. Persistence of tinnitus can reflect deteriorating audition. Vertigo control and hearing preservation are independent outcomes of EDB surgery, making it desirable as an early intervention for refractory MD cases. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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13 pages, 593 KiB

Open AccessArticle

Vestibular Loss in Children Affected by LVAS and IP2 Malformation and Operated with Cochlear Implant

by Åsa Bonnard, Eva Karltorp and Luca Verrecchia

Audiol. Res. 2023, 13(1), 130-142; https://doi.org/10.3390/audiolres13010013 - 9 Feb 2023

Cited by 1 | Viewed by 1853

Abstract

This is a single center cohort study regarding the prevalence of vestibular loss in hearing impaired children affected by large vestibular aqueduct syndrome (LVAS) with incomplete cochlear partition malformation type II (IP2), fitted with cochlear implant (CI). Twenty-seven children received CI operations at 0.4–13 years on one or both ears and tested for vestibular loss with head impulse test, video head impulse test, mini ice-water test and cervical VEMP. Vestibular loss was found in 19% of operated ears and in 13.9% of non-operated ears. The difference was not statistically significant and was not significantly modified by age at implantation, age at testing, sex, presence of SLC26A4 gene mutation or bilaterality. However, the presence of anatomic anomalies at the level of the vestibulum or semicircular canals was significantly associated with a higher incidence of vestibular loss in CI operated children but not in those non-operated. No other factors, such as the surgical access, the electrode type, the presence of Gusher perioperatively, or post-operative vertigo modified significantly the prevalence of vestibular loss. In conclusion, LVAS/IP2 appears to be the major determinant of vestibular loss in these children, with a less obvious impact of CI, excluding the cases with vestibulum/canal anomalies: this group might have a higher risk for vestibular loss after CI surgery. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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Review

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9 pages, 236 KiB

Open AccessReview

Audio-Vestibular Evaluation of Pediatric Pseudo-Conductive Hearing Loss: Third Window Syndromes

by Gorkem Ertugrul, Aycan Comert and Aysenur Aykul Yagcioglu

Audiol. Res. 2024, 14(5), 790-798; https://doi.org/10.3390/audiolres14050066 - 6 Sep 2024

Viewed by 1057

Abstract

Conductive hearing loss caused by external or middle ear problems prevents the transmission of sound waves from the external auditory canal to the cochlea, and it is a common condition, especially in pediatric patients aged 1–5 years. The most common etiological factors are otitis media and cerumen during childhood. In some patients, external and middle ear functions and structures may be normal bilaterally despite the air-bone gap on the audiogram. This condition, which is often a missed diagnosis in children, is defined as a pseudo-conductive hearing loss (PCHL) caused by third window syndromes (TWSs) such as semicircular canal dehiscence, inner ear malformations with third window effect, and perilymphatic fistula. In this review of the literature, the authors emphasize the pitfalls of pediatric audio-vestibular evaluation on TWSs as well as the key aspects of this evaluation for the differential diagnosis of PCHL brought on by TWSs. This literature review will provide audiologists and otologists with early diagnostic guidance for TWSs in pediatric patients. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

Other

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4 pages, 159 KiB

Open AccessPerspective

Issues Concerning the Mechanisms of Bone Conduction

by Miriam Geal-Dor, Cahtia Adelman and Haim Sohmer

Audiol. Res. 2024, 14(5), 840-843; https://doi.org/10.3390/audiolres14050070 - 17 Sep 2024

Viewed by 745

Abstract

Air and bone conduction thresholds are used to differentiate between conductive and sensori-neural hearing losses because bone conduction is thought to bypass the conductive apparatus, directly activating the inner ear. However, the suggested bone conduction mechanisms involve the outer and middle ears. Also, normal bone conduction thresholds have been reported in cases of lesions to the conduction pathway. Therefore, further investigation of bone conduction mechanisms is required. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

12 pages, 1318 KiB

Open AccessCase Report

Low-Frequency Air–Bone Gap and Pulsatile Tinnitus Due to a Dural Arteriovenous Fistula: Considerations upon Possible Pathomechanisms and Literature Review

by Andrea Tozzi, Andrea Castellucci, Giuseppe Ferrulli, Salvatore Martellucci, Pasquale Malara, Cristina Brandolini, Enrico Armato and Angelo Ghidini

Audiol. Res. 2023, 13(6), 833-844; https://doi.org/10.3390/audiolres13060073 - 1 Nov 2023

Cited by 1 | Viewed by 2498

Abstract

Low-frequency air–bone gap (ABG) associated with pulsatile tinnitus (PT) and normal impedance audiometry represents a common finding in patients with third window syndromes. Other inner disorders, including Meniere’s disease (MD), perilymphatic fistula and intralabyrinthine schwannoma, might sometimes result in a similar scenario. On the other hand, PT is frequently associated with dural arteriovenous fistula (DAVF), while conductive hearing loss (CHL) is extremely rare in this clinical setting. A 47-year-old patient was referred to our center with progressive left-sided PT alongside ipsilateral fullness and hearing loss. She also experienced headache and dizziness. Otoscopy and video-oculographic examination were unremarkable. Conversely, a detailed instrumental audio-vestibular assessment revealed low-frequency CHL with normal impedance audiometry, slight left-sided caloric weakness, slightly impaired vestibular-evoked myogenic potentials on the left and normal results on the video-head impulse test, consistent with an MD-like instrumental profile. Gadolinium-enhanced brain MRI revealed an early enhancement of the left transverse sinus, consistent with a left DAVF between the left occipital artery and the transverse sinus, which was then confirmed by angiography. A trans-arterial embolization with Onyx glue was performed, resulting in a complete recession of the symptoms. Post-operatively, the low-frequency ABG disappeared, supporting the possible role of venous intracranial hypertension and abnormal pressure of inner ear fluids in the onset of symptoms and offering new insights into the pathomechanism of inner ear CHL. Full article

(This article belongs to the Special Issue Inner Ear Conductive Hearing Loss: Current Studies and Controversies)

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