Conductive Hearing Loss in Children.

A variety of congenital and acquired disorders result in pediatric conductive hearing loss. Malformations of the external auditory canal are invariably associated with malformations of the middle ear space and ossicles. Isolated ossicular malformations are uncommon. Syndromes associated with external and middle ear malformations are frequently associated with abnormal development of first and second pharyngeal arch derivatives. Chronic inflammatory disorders include cholesteatoma, cholesterol granuloma, and tympanosclerosis.

Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features.

OBJECTIVES: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. METHODS: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children's Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. RESULTS: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. CONCLUSION: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

Cone-Beam CT Compared to Multi-Slice CT for the Diagnostic Analysis of Conductive Hearing Loss: A Feasibility Study.

OBJECTIVES: Multislice computed tomography (MSCT) is commonly used as a diagnostic tool for patients with a conductive hearing loss. Recent studies indicate that cone-beam computed tomography (CBCT) may be used as a low-radiation dose alternative for temporal bone imaging. This study compares image quality and radiation dose between CBCT and MSCT when assessing anatomical landmarks related to conductive hearing loss. MATERIALS AND METHODS: Five human cadaver heads (10 ears) were imaged on the NewTom 5G CBCT and the Discovery CT750 HD MSCT. Visibility of 16 anatomical landmarks of the middle and inner ear was assessed by two observers on a 4-point Likert scale. Furthermore, effective radiation dose was compared, and contrast-to-noise ratio and spatial resolution were measured with a phantom head. RESULTS: Image quality of CBCT was assessed as superior to MSCT. Effective radiation dose of the high-resolution CBCT protocol was 30.5% of the clinical MSCT dose. High-resolution CBCT was reported as having a higher spatial resolution and superior contrast-to-noise perception in comparison with MSCT. CONCLUSION: High-resolution CBCT was evaluated as superior to MSCT in the assessment of structures related to conductive hearing loss. Furthermore, CBCT imaging resulted in a considerably lower effective radiation dose.

High Resolution Computed Tomography (HRCT) Imaging Findings of Oval Window Atresia with Surgical Correlation.

INTRODUCTION: Isolated oval window atresia (OWA) is a rare cause of congenital conductive middle ear deafness and may be overlooked owing to the normal appearance of the external ear. This anomaly has been previously described, although the published numbers with both imaging and surgical findings are few. Our aim is to correlate the imaging features of OWA with intraoperative findings. MATERIALS AND METHODS: This is a single-centre retrospective evaluation of patients who were diagnosed with OWA and who received surgery from January 1999 to July 2006. No new case was diagnosed after 2006 to the time of preparation of this manuscript. High resolution computed tomography (HRCT) imaging of the temporal bones of the patients were retrospectively evaluated by 2 head and neck radiologists. Images were evaluated for the absence of the oval window, ossicular chain abnormalities, position of the facial nerve canal, and other malformations. Imaging findings were then correlated with surgical findings. RESULTS: A total of 9 ears in 7 patients (two of whom with bilateral lesions) had surgery for OWA. All patients had concomitant findings of absent stapes footplate with normal, deformed or absent stapes superstructure and an inferiorly displaced facial nerve canal. HRCT was sensitive in identifying OWA and associated ossicular chain and facial nerve abnormalities, which were documented surgically. CONCLUSION: OWA is a rare entity that can be diagnosed with certainty on HRCT, best visualised on coronal plane. Imaging findings of associated middle ear abnormalities, position of the facial nerve canal, which is invariably mal-positioned, and associated deformity of the incus are important for presurgical planning and consent.

A Case Series of X-Linked Deafness-2 with Sensorineural Hearing Loss, Stapes Fixation, and Perilymphatic Gusher: MR Imaging and Clinical Features of Hypothalamic Malformations.

X-linked deafness-2 (DFNX2) is an X-linked recessive disorder characterized by profound sensorineural hearing loss and a pathognomonic temporal bone deformity. Because hypothalamic malformations associated with DFNX2 have been rarely described, we aimed to further describe these lesions and compare them with features of a nonaffected population. All patients diagnosed with DFNX2 between 2006 and 2019 were included and compared with age-matched patients with normal MR imaging findings and without hypothalamic dysfunction. MR imaging features differing between groups were selected to help identify DFNX2. Sensitivity and specificity were calculated for these features. Agreement among 3 radiologists was quantified using the index κ. Information on the presence or absence of gelastic seizures, precocious puberty, or delayed puberty was also gathered. We selected distinctive MR imaging features of hypothalamic malformations in DFNX2. The feature selected on axial T2 images was the folded appearance of the ventromedial hypothalamus (sensitivity, 100%; specificity, 95.8%) characterized by an abnormal internal/external cleft (sensitivity, 100%; specificity, 95.7%). On coronal T2, the first distinctive feature was a concave morphology of the medial eminence (sensitivity, 100%; specificity, 97.1%), the second feature was at least 1 hypothalamic-septum angle ≥90° (sensitivity, 90%; specificity, 72.5%), and the third feature was a forebrain-hypothalamic craniocaudal length of ≥6 mm (sensitivity, 70%; specificity, 79.7%). Clinical features were also distinctive because 9 patients with DFNX2 did not present with gelastic seizures or precocious puberty. One patient had delayed puberty. The κ index and intraclass correlation coefficient ranged between 0.78 and 0.95. Imaging and clinical features of the hypothalamus suggest that there is a hypothalamic malformation associated with DFNX2. Early assessment for pubertal delay is proposed.

New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review.

Incomplete partition type III, also referred to as X-linked deafness, is a rare genetic inner ear malformation. Its characteristic CT findings, including bulbous dilation of the internal auditory canal and absence of the modiolus with the interscalar septa present, have been well-recognized. In this series of 19 cases, we report the abnormalities of the vestibule and semicircular canals and provide a comprehensive description of their CT and MR imaging findings. The inner ear malformations in incomplete partition type III were bilateral and basically symmetric, with involvement of the internal auditory canal, nerve canals in the fundus, cochlea, vestibule, semicircular canals, vestibular aqueduct, otic capsule, round window, oval window, and stapes. An irregular vestibule with a cystic appearance is also a distinctive imaging feature, which could be seen in about 90% of our patients, with a cystic appearance of the semicircular canals present in nearly half of the cases.

Implantation of bone-anchored hearing device using a three-dimensional template in a child.

BACKGROUND: Implantation of bone-anchored hearing devices is performed to improve hearing in patients with chronic suppurative otitis media who cannot wear a conventional hearing aid. The surgical procedure can be safely performed in children aged over five years. CASE REPORT: A 15-year-old patient with bilateral chronic suppurative otitis media and conductive hearing loss underwent the procedure to implant a bone-anchored hearing device but was found to have skull thickness of less than 2.5 mm and the procedure was abandoned. A computed tomography scan of the skull was undertaken and a three-dimensional template was reconstructed to identify appropriate thickness of the skull to implant the abutment during a second procedure. CONCLUSION: Bone-anchored hearing devices can be implanted by prior imaging and using a template to identify the area of appropriate skull thickness to implant the abutment safely.

Isolated malleus fixation: A pediatric case series.

OBJECTIVES: The goal of this review was to review our series of isolated malleus fixation in pediatric patients, a rare entity causing conductive hearing loss. Malleolar fixation is poorly described in this patient population. METHODS: A retrospective review of pediatric tympanoplasties by the senior author over a four-year period was performed. Only cases with isolated fixation of the malleus were reviewed. Primary outcome of interest was post-operative hearing. Paired t-tests were used to calculate pre- and post-operative hearing outcomes. RESULTS: Five cases were analyzed. Mean age at time of surgery was 9.1 years (range 4.4-16.0 years). Average follow-up after surgery was 13.9 months (range 4.4-31.2 months). Patients were otherwise healthy and typically presented after a failed school hearing test despite previously good hearing. Three out of five cases showed radiographic evidence of bony fixation (60%) on computerized tomography (CT). Otoscopy was unremarkable in all cases. Average procedure time was 41.2 min and consisted of transcanal tympanoplasty with excision of fixed bony segment. A significant improvement in both pre- and post-operative air bone gaps was observed (p = 0.005)., with average ABG of 14.75 dB. CONCLUSIONS: Isolated pediatric malleolar fixation is an uncommon cause of pediatric conductive hearing loss. CT scan is useful for identifying this abnormality, and surgical correction results in improved post-operative hearing outcomes, potentially obviating the need for hearing amplification.

Conductive hearing loss with a "dry middle ear cleft"-A comprehensive pictorial review with CT.

Conductive hearing loss (CHL) commonly results from middle ear fluid and inflammation (otitis media). Less commonly in patients with CHL, the middle ear cleft is well aerated or 'dry' with absence of soft tissue or fluid clinically and on imaging. There are numerous causes for this but they can be clinically challenging to diagnose. This pictorial review aims to illustrate and discuss the CT features of both common and less common causes of CHL in patients with a "dry middle ear cavity".

Endoscopic optical coherence tomography with wide field-of-view for the morphological and functional assessment of the human tympanic membrane.

An endoscopic optical coherence tomography (OCT) system with a wide field-of-view of 8 mm is presented, which combines the image capability of endoscopic imaging at the middle ear with the advantages of functional OCT imaging, allowing a morphological and functional assessment of the human tympanic membrane. The endoscopic tube has a diameter of 3.5 mm and contains gradient-index optics for simultaneous forward-viewing OCT and video endoscopy. The endoscope allows the three-dimensional visualization of nearly the entire tympanic membrane. In addition, the oscillation of the tympanic membrane is measured spatially resolved and in the frequency range between 500 Hz and 5 kHz with 125 Hz resolution, which is realized by phase-resolved Doppler OCT imaging during acoustical excitation with chirp signals. The applicability of the OCT system is demonstrated in vivo. Due to the fast image acquisition, structural and functional measurements are only slightly affected by motion artifacts.

Association of Hearing Loss and Otologic Outcomes With Fibrous Dysplasia.

Importance: Fibrous dysplasia (FD) and McCune-Albright syndrome (MAS) are rare bone and endocrine disorders in which expansile fibro-osseous lesions result in deformity, pain, and functional impairment. The effect of FD on hearing and otologic function has not been established. Objectives: To characterize audiologic and otologic manifestations in a large cohort of individuals with FD/MAS and to investigate potential mechanisms of hearing loss. Design, Setting, and Participants: In this natural history study, individuals with craniofacial FD seen at a clinical research center underwent clinical, biochemical, computed tomographic, audiologic, and otolaryngologic evaluations. Main Outcomes and Measures: Clinical and radiologic features associated with hearing loss and otologic disease were evaluated. Conductive hearing loss was hypothesized to be associated with narrowing of the external auditory canal (EAC), FD involving the epitympanum, and FD crowding the ossicular chain. Sensorineural hearing loss was hypothesized to be associated with FD affecting the internal auditory canal (IAC) and otic capsule. Results: Of the 130 study participants with craniofacial FD who were evaluated, 116 (89.2%) had FD that involved the temporal bone (median age, 19.6 years; range, 4.6-80.3 years; 64 female [55.2%]), whereas 14 (10.8%) had craniofacial FD that did not involve the temporal bone. Of the 183 ears with temporal bone FD, hearing loss was identified in 41 ears (22.4%) and was conductive in 27 (65.9%), sensorineural in 12 (29.3%), and mixed in 2 (4.9%). Hearing loss was mild and nonprogressive in most participants. Whereas EACs were narrower in ears with FD (mean difference [MD], 0.33 mm; 95% CI, 0.11-0.55 mm), this finding was associated with conductive hearing loss in only 4 participants. Fibrous dysplasia crowding of the ossicles was associated with conductive hearing loss (odds ratio [OR], 5.0; 95% CI, 2.1-11.6). The IAC length was not different between ears with and without FD (MD, -0.37; 95% CI, -0.95 to 0.211); however, canals were elongated in ears with sensorineural hearing loss (MD, -1.33; 95% CI, -2.60 to -0.07). Otic capsule involvement was noted in only 4 participants, 2 of whom had sensorineural hearing loss. Both MAS-associated growth hormone excess (OR, 3.1; 95% CI, 1.3-7.5) and neonatal hypercortisolism (OR, 11; 95% CI, 2.5-55) were associated with an increased risk of hearing loss . Conclusions and Relevance: Hearing loss in craniofacial FD is common and mild to moderate in most individuals. It typically arises from FD crowding of the ossicular chain and elongation of the IAC, whereas EAC stenosis and otic capsule invasion are less common causes. Individuals with craniofacial FD should undergo otolaryngologic evaluation and monitoring, including assessment to identify those with high-risk features.

High-Resolution Computed Tomography Imaging in Conductive Hearing Loss: What to Look for?

Conductive hearing loss (CHL) is caused by the disruption of the sound conductive chain that, in turn, may be due to diseases of the external and middle ear. High-resolution computed tomography (CT) is the imaging modality of choice to evaluate CHL because of its excellent spatial resolution. Along with clinical and otoscopic findings, CT also helps in the diagnosis and preoperative planning. This pictorial review aims to illustrate the CT features of common conditions causing CHL that may arise from the external and middle ear, as well as highlights some of the key imaging features that are helpful in management.

Transtympanic pseudoaneurysm of the internal carotid artery complicating a myringotomy in a four-year old child: Case report and literature review.

We report the first case of a transtympanic iatrogenic internal carotid artery (ICA) pseudoaneurysm diagnosed in a 4-year-old child following a myringotomy. An endovascular treatment with a covered-stent was decided; spontaneous thrombosis was found during the therapeutic arteriography, and the procedure was aborted. Otoscopy and computed tomography (CT) scan monitoring showed a prolonged thrombosis and the disappearance of the pseudoaneurysm 18months after the diagnostic arteriography. Based on literature review, endovascular techniques seem to be preferred to the surgical approach for treatment of intrapetrous ICA pseudoaneurysm, however clinical and CT scan monitoring may also be a valid option.

The relationship between post-traumatic ossicular injuries and conductive hearing loss: A 3D-CT study.

PURPOSE: After a trauma, the conductive ossicular chain may be disrupted by ossicular luxation or fracture. Recent developments in 3D-CT allow a better understanding of ossicular injuries. In this retrospective study, we compared patients with post-traumatic conductive hearing loss (CHL) with those referred without CHL to evaluate the relationship between ossicular injuries and CHL. We also assessed the added value of 3D reconstructions on 2D-CT scan to detect ossicular lesions in patients surgically managed. METHODS: The CT scans were performed using a 40-section spiral CT scanner in 49 patients with post-traumatic CHL (n=29) and without CHL (n=20). Three radiologists performed independent blind evaluations of 2D-CT and 3D reconstructions to detect ossicular chain injury. We used the t-test to explore differences regarding the number of subjects with ossicular injury in the two groups. We also estimated the diagnostic accuracy and the inter-rater agreement of the 3D-CT reconstructions associated to 2D-CT scan. RESULTS: We identified ossicular abnormality in 14 patients out of 29 and in one patient out of 20 in the CHL and non-CHL groups respectively. There was a significant difference regarding the number of subjects with ossicular lesions between the two groups (P≤0.01). The diagnostic sensitivity of 3D-CT reconstructions associated with 2D-CT ranged from 66% to 100% and the inter-reader agreement ranged from 0.85 to 1, depending of the type of lesion. CONCLUSION: The relationship between ossicular lesion and the presence of CHL tightly correlated. 3D-CT reconstructions of the temporal bone are useful to assess patients in a post-traumatic context.

Vascular mapping of the retroauricular skin - proposal for a posterior superior surgical incision for transcutaneous bone-conduction hearing implants.

BACKGROUND: Passive transcutaneous osseointegrated hearing implant systems have become increasingly popular more recently. The area over the implant is vulnerable due to vibration and pressure from the externally worn sound processor. Good perfusion and neural integrity has the potential to reduce complications. The authors' objective was to determine the ideal surgical exposure to maintain perfusion and neural integrity and decrease surgical time as a result of reduced bleeding. METHODS: The vascular anatomy of the temporal-parietal soft tissue was examined in a total of 50 subjects. Imaging diagnostics included magnetic resonance angiography in 12 and Doppler ultrasound in 25 healthy subjects to reveal the arterial network. Cadaver dissection of 13 subjects formed the control group. The prevalence of the arteries were statistically analyzed with sector analysis in the surgically relevant area. RESULTS: The main arterial branches of this region could be well identified with each method. Statistical analysis showed that the arterial pattern was similar in all subjects. The prevalence of major arteries is low in the upper posterior area though large in proximity to the auricle region. CONCLUSIONS: Diverse methods indicate the advantages of a posterior superior incision because the major arteries and nerves are at less risk of damage and best preserved. Although injury to these structures is rare, when it occurs, the distal flow is compromised and the peri-implant area is left intact. Hand-held Doppler is efficient and cost-effective in finding the best position for incision, if necessary, in subjects with a history of surgical stress to the retroauricular skin. TRIAL REGISTRATION: This was a non-interventional study.

Novel syndrome with conductive hearing loss and congenital glaucoma in three generations.

The objective of this paper was to describe the clinical and otological findings in multiple members of a family with congenital glaucoma, cardiac anomaly, and conductive hearing loss due to ossicular chain anomalies. We performed a retrospective review of the medical charts and otological materials of multiple members of the same family. Congenital glaucoma and hearing loss were inherited by the proband and her daughter, son, and mother, suggesting autosomal dominant inheritance. The son and daughter also showed atrial septal defects. Exploratory tympanotomies revealed anomalies of the long process of the incus in the proband and her daughter, and tympanoplasty improved hearing loss in both patients. This represents the first description of coexisting congenital glaucoma and conductive hearing loss due to ossicular chain anomalies in multiple members of a single family.

Case 226: Oval Window Atresia.

A 6-year-old girl presented with bilateral hearing loss. Her otologic, birth, and family histories were limited, given that she was adopted, but her parents reported that she had had difficulty hearing and speaking ever since they adopted her at 2 years of age. Her parents denied a history of acute otitis media, otorrhea, otalgia, vertigo, autophony, or tinnitus since her adoption. At 2.5 years of age, a diagnosis of hearing loss was made, and she was given hearing aids. Her parents believed that she had been doing well with both receptive and expressive language since she had received the hearing aids. At examination, she had small bilateral preauricular skin tags and normal pinna. Her external auditory canals were of a normal caliber bilaterally, with no otorrhea or lesions. The tympanic membranes were translucent and mobile at pneumatic otoscopy. There was no evidence of a middle ear lesion, nor was there a Schwartz sign. She had no nystagmus or vertigo at pneumatic otoscopy. Audiometry was performed and revealed moderate to severe conductive hearing loss bilaterally, with a mixed component present at 2000 KHz. She had normal bilateral middle ear pressure at tympanometry. Thin-section computed tomography (CT) of the temporal bone was performed.

Imaging of Conductive Hearing Loss With a Normal Tympanic Membrane.

OBJECTIVE: This article presents an approach to imaging conductive hearing loss in patients with normal tympanic membranes and discusses entities that should be checked as the radiologist evaluates this potentially complicated issue. CONCLUSION: Conductive hearing loss in a patient with a normal tympanic membrane is a complicated condition that requires a careful imaging approach. Imaging should focus on otosclerosis, and possible mimics and potential surgical considerations should be evaluated. The radiologist should examine the ossicular chain and the round window and keep in mind that a defect in the superior semicircular canal can disturb the hydraulic integrity of the labyrinth.