Brachio-Oto-Renal Syndrome: CT Imaging and Intraoperative Diagnostic Findings.

Background: Most previous studies are separate dosimetric analyses of conductive or sensorineural hearing loss, and they are not conducive to a comprehensive assessment of auditory radiation damage.Aims/objectives: Our study aimed to evaluate the long-term incidence of sensorineural hearing loss (SNHL) or conductive hearing loss (CHL) in patients with nasopharyngeal carcinoma (NPC) after intensity-modulated radiation therapy (IMRT), and to investigate the relationship between SNHL or CHL and patient factors, treatment-related factors, and radiation dose parameters.Material and methods: Seventy patients (117 ears) with NPC, who were also treated with IMRT in our hospital from 2006 to 2014, were retrospectively analyzed. Radiation doses to the Eustachian tube (ET), middle ear (ME), cochlear (Co), and internal auditory canal (IAC) were assessed. Pure tone audiometry and impedance audiometry were performed before and during the follow-up period. The relationships between low-frequencies (0.5–2 kHz) or high-frequency (4 kHz) SNHL/CHL and radiotherapy dose parameters were analyzed.Results: Of the 117 ears studied, 7.69% had low-frequency SNHL, 35.9% had high-frequency SNHL, 23.93% had low-frequency CHL, and 18.80% had high-frequency CHL. The incidence of high-frequency CHL was higher in the T4 group than in the T (1–3) group (p < .05). When IAC Dmax > 42.13 Gy or IAC Dmean > 32.71 Gy, the risk of high-frequency SNHL increased in NPC patients. When ME Dmax > 44.27 Gy, ME Dmean > 29.28 Gy, or ET Dmax > 57.23 Gy, the risk of high-frequency CHL in NPC patients increased.Conclusions and significance: SNHL and CHL remain common ear complications after IMRT for NPC. IAC Dmax, IAC Dmean, ME Dmax, ME Dmean, and ET Dmax all need to be carefully considered during the IMRT treatment protocol.

On the basis of strong research, universal newborn screening should be performed before age 1 month with repeat or follow-up testing for those who do not pass performed before age 3 months and intervention started before age 6 months. On the basis of strong research and consensus statement, tympanostomy tubes should be considered for individuals with bilateral persistent middle ear effusion for 3 months or greater and a documented conductive hearing loss. On the basis of consensus statement, all children with suspected hearing loss should have an age appropriate hearing test. On the basis of strong research, the most common form of congenital hearing loss is genetic. Most of this is nonsyndromic hearing loss.

Perioperative Hearing Impairment

A40-year-old patient presented to the clinic with a history of persistent aural fullness and exacerbating hearing loss on the right. He denied tinnitus, otalgia, headaches, vertigo, facial nerve symptoms, or trauma. His medical history is significant for surgery and radiation for olfactory neuroblastoma (tumor of the anterior skull base above the nose) and a sphenoid mucocele that was endoscopically excised one month before presentation. Physical examination demonstrated bulging of the tympanic membrane and a purple-colored tympanic membrane (Figure 1). The left ear was normal. The audiogram demonstrated a mixed moderately severe to severe hearing loss on the right with a 20-30 dB air-bone gap and mild sensorineural hearing loss on the left (Figure 2).Figure 1: Image of patient’s ear. Arrows point to the tympanic membrane. Clinical Consultation, purple ear drum, middle ear cholesterol granuloma.Figure 2: Patient’s audiogram. Clinical Consultation, purple ear drum, middle ear cholesterol granuloma.Figure 3: Axial (horizontal) T1 fat saturated without (A) and with (B) gadolinium MRI showing hyperintensity (brighter than brain) in the middle ear, mastoid, and Eustachian tube. Clinical Consultation, purple ear drum, middle ear cholesterol granuloma.Figure 4: Coronal (parallel to the face) T2 MRI showing middle ear fluid is slightly hyperintense (brighter) compared with the brain. Clinical Consultation, purple ear drum, middle ear cholesterol granuloma.Figure 5: Axial (horizontal) CT of temporal bones showing bulging of the tympanic membrane. Clinical Consultation, purple ear drum, middle ear cholesterol granuloma.Figure 6: Axial (horizontal) T2 MRI from 14 years prior showing fluid in Eustachian tube but middle ear with no fluid or cholesterol granuloma. Clinical Consultation, purple ear drum, middle ear cholesterol granuloma.DIAGNOSIS: MIDDLE EAR CHOLESTEROL GRANULOMA Our patient presented with a purplish-red mass bulging from the tympanic membrane. At first, this lesion is suspected to be a hypervascular mass. Vascular masses in the middle ear are uncommon and usually asymptomatic; however, patients may present with nonspecific symptoms such as pulsatile tinnitus, aural fullness, otalgia, and hearing loss. It is important to make the diagnosis before any middle ear surgery to avoid significant blood loss. The differential diagnosis can range from a dehiscent jugular bulb, aberrant internal carotid artery, and glomus tympanicum, to cholesterol granuloma and hemorrhage. A dehiscent high-riding jugular bulb is the most commonly encountered cause for a blue/light purple mass under the tympanic membrane usually in the posterior inferior aspect of the middle ear. It would be unusual for it to be present behind the entire tympanic membrane. The internal jugular vein constitutes the continuum of the dural venous sinuses in the neck and lies below the hypotympanum. However, in rare instances, the bony jugular fossa can be absent, and a jugular bulb may extend above the level of the inferior tympanic annulus and show an indentation in the middle ear. It’s usually asymptomatic. When symptomatic, a high-riding jugular bulb may cause pulsatile tinnitus, conductive hearing loss, and vertigo. Conductive hearing loss can be due to the mass loading of the tympanic membrane, the ossicles, or blockage of the round window. A high-resolution computed tomography (CT) scan usually demonstrates dehiscence of the right bony septum since the dural sinuses and jugular vein are larger on the right. 1 Surgical repair can be performed to improve the conductive hearing loss. Another possible cause of a purplish-red mass behind the tympanic membrane is an aberrant internal carotid artery (ICA). It’s a rare vascular anomaly to be seen in the middle ear (< 1%). The dehiscence of the cervical part of the ICA can be due to an error in embryogenesis (absence of the carotid canal and lateral posterior displacement of the carotid due to the persistence of embryonic vasculature) or acquired factors such as malignancies or skull base surgeries. Based on otoscopic findings and non-specific clinical presentation, an aberrant ICA often will be present in the anterior middle ear (where the carotid artery is located) and can appear purple, white, or red depending on the prominence and dominance of the overlying vasa vasorum (small blood vessels that feed the wall of the carotid artery). When present low in the middle ear, it can mimic a glomus tympanicum – a highly vascular benign tumor of the middle ear. However, a CT scan of the temporal bone helps avoid misdiagnosis, since, in the case of a glomus tumor, the size and aspect of the carotid canal are normal. 2 In addition, glomus tympanicum usually develops later in life, around the fifth to sixth decades and present with pulsatile tinnitus and conductive hearing loss. Another consideration for a purple tympanic membrane is hemorrhage in the middle ear that can be due to direct trauma (e.g., temporal bone fracture) or barotrauma. Barotrauma can occur from aggressive attempts at ear popping with severe Eustachian tube dysfunction, barotrauma following a flight. The patient’s history can usually give the clinician the information needed to understand the etiology. Usually, barotrauma will present with bleeding just anterior to the malleus but can include the entire middle ear. Cholesterol granulomas are cystic lesions commonly involving the petrous apex. Rarely, they can present behind the tympanic membrane as a purple or blue mass. First described by Paparella and Lim 3, two theories attempted to explain the development of cholesterol granuloma. The obstruction-vacuum theory states that the negative pressure due to Eustachian tube dysfunction or pneumatized air cells causes the extravasation of intravascular fluid which leads to chronic mucosal edema and breakdown and subsequent repeated episodes of bleeding. The hemorrhage incites an inflammatory reaction and the formation of cysts surrounding degrading blood products (cholesterol). The cysts gradually expand following a repeat of the described cycle. On the other hand, the exposed marrow hypothesis (primarily discussed for petrous apex cholesterol granuloma) states that the pneumatization of the bone expose the bone marrow to the hyperplastic mucosa. The coaptation of the bone marrow and mucosa will bleed, which will result in the inflammatory reaction and the formation of cysts. 4 Symptoms associated with cholesterol granuloma include hearing loss, tinnitus, aural fullness, headaches, and vestibular symptoms depending on how they expand and what structures are involved. Cholesterol granuloma can be identified by its high signal intensity (brighter than the brain) on FLAIR, T1-, and T2-weighted magnetic resonance imaging (MRI). On CT scan, middle ear cholesterol granulomas tend to not be as aggressive in growth and destruction compared with the petrous apex cholesterol granulomas that can be more aggressive. In order to differentiate the aforementioned lesions, an MRI and CT scan of the temporal bone should be obtained. The patient’s MRI showed hyperintense (brighter than the brain) fluid in the right middle ear on T1 and T2-weighted images of the temporal bones (Figures 345). The temporal bone CT showed fluid in the right middle ear and mastoid (gray color) and bulging of the tympanic membrane without bony destruction. The absence of bony erosion indicates that a tumor is less likely to be present (Figure 6). These findings indicate that the bulging mass is a cholesterol granuloma. Based on the history of our patient, it is most probably due to Eustachian tube dysfunction caused by his previous surgery and radiation for his olfactory neuroblastoma. Cholesterol granuloma can be managed by surgical drainage to prevent further expansion. When the cyst is symptomatic and located in the petrous apex, the treatment is challenging due to its proximity or erosion of critical structures (cochlea, carotid artery, and jugular vein). Its surgical drainage can be accomplished via infracochlear, transmastoid, middle fossa, transarcuate, or retrolabyrinthine approach. Resection is generally not attempted as drainage of the cholesterol granuloma suffices to stop its growth. 5 On the other hand, in case of a middle ear cholesterol granuloma, myringotomy and tube placement in the office treats the problem. The patient underwent a myringotomy in the posterior inferior quadrant and tube placement in the office. Brown (motor oil-like) fluid was aspirated from the middle ear; thus relieving the aural pressure and normalizing the tympanic membrane. BONUS ONLINE VIDEOS: VISUAL DIAGNOSIS Read this month’s Clinical Consultation case, then watch the accompanying videos from Hamid R. Djalilian, MD, to review the patient’s imaging for yourself. Video 1. Axial (horizontal) T1 fat saturated MRI showing the hyperintensity in the middle ear and Eustachian tube. Video 2. Coronal (parallel to the face) T2 MRI showing the fluid consistency on T2 images and the mucocele in sinus. Video 3. Axial (horizontal) T1 fat saturated post-gadolinium MRI showing the subtle inflammatory reaction around the cholesterol granuloma. Video 4. Axial (horizontal) CT of temporal bones showing no destruction of the mastoid. Video 5. Coronal (parallel to the face) CT of temporal bones showing no ossicular destruction. Video 6. Axial (horizontal) T2 MRI from 2008 showing no cholesterol granuloma is present. Watch the patient videos online at thehearingjournal.com.

Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

View Video S1 Laryngoscope, 131:E961–E965, 2021

Tympanosclerosis may involve the tympanic membrane, the ossicles, as well as the oval and round window niche, respectively.The surgical treatment of the obliterated oval window niche is most challenging.Beside stapesplasty, vibroplasty with coupling the floating mass transducer (FMT) onto the round window niche and into a new, so-called third window is indicated.In the latter situation, drilling a hole into the promontorium is necessary to couple the FMT close to the membranous endosteum.Any damage of the membranous inner ear needs to be avoided.The question was whether OCT is useful to identify the endosteum and to provide microanatomical information about the round window niche.OCT was carried out on human temporal bone preparations in which a third window was drilled, while leaving the membranous labyrinth and the fluid-filled inner ear intact and removing the overhang of the round window niche.A specially equipped operating microscope with integrated OCT prototype (spectral-domain-OCT) was used.The OCT images and 3D reconstructions demonstrate the usefulness of OCT to measure the drilling cavity, to visualize the inner ear structures, and to obtain microanatomical information about the round and oval window niche.These findings may have an impact on stapes surgery, on cochlea implantation, and on vibroplasty with coupling the FMT onto the round and third window.OCT-guided drilling allows identification of the intact inner ear more precisely.

An optical coherence tomography study for imaging the round window niche and the promontorium tympani Tympanosclerosis may involve the tympanic membrane, the ossicles, and the oval and round window niche, respectively. The surgical treatment of the obliterated oval window niche is most challenging. Beside stapesplasty, vibroplasty coupling the floating mass transducer (FMT) onto the round window niche and into a new, so-called third window is indicated. In the latter situation, drilling a hole into the promontorium is necessary to couple the FMT close to the membranous endosteum. Damage of the membranous inner ear must be avoided. The question was whether OCT is useful to identify the endosteum and to provide microanatomical information of the round window niche. OCT was carried out on human temporal bone preparations, in which a third window was drilled leaving the membranous labyrinth and the fluid-filled inner ear intact and the overhang of the round window niche was removed. An especially equipped operating microscope with integrated OCT prototype (spectral-domain-OCT) was used. The OCT images and 3D reconstructions demonstrate the usefulness of OCT to measure the drilling cavity, to visualize the inner ear structures, and to obtain microanatomical information of the round and oval window niche. These findings may have an impact on stapes surgery, on cochlea implantation, and on vibroplasty coupling the FMT onto the round and third window. OCTguided drilling allows for more precise identification of the intact inner ear.

To evaluate modified coupling techniques of the Vibrant Soundbridge system in patients with mixed and conductive hearing loss and to compare it with conventional vibroplasty. Retrospective study. Two different groups were evaluated: 1) nine cases of conventional incus vibroplasty in comparison with 2) nine patients with modified coupling of the floating mass transducer. In the modified coupling approach, the vibrant floating mass transducer was attached to 1) the stapes/oval window, 2) the round window, or 3) the drilled promontory bone (promontory fenestration window). In three patients, an additional ossiculoplasty was performed. Preoperative and postoperative aided and unaided pure-tone and free-field audiometry and Freiburg monosyllabic word test were used to assess hearing outcome. Functional hearing gain obtained in patients with mixed and conductive hearing loss who underwent modified coupling was 39 dB. Patients with pure sensorineural hearing loss who received conventional incus coupling showed a functional hearing gain of 25 dB. Average functional gain was 41 dB in the oval window group, 45 dB in the round window group, and 30 dB in the promontory fenestration window group. Word recognition test revealed an average improvement of 51% and 21% in the modified and in the conventional approach, respectively. Modified vibroplasty is a safe and effective treatment for patients with conductive and mixed hearing loss. Coupling the floating mass transducer to the promontory bone (promontory fenestration window) is a viable option in chronically disabled ears if oval and round window coupling is not possible. 4.

To identify primary bony cysts of the temporal bone. A single case of a woman presenting with unilateral bulging of the temporoparietal cranium, progressive stenosis of the external auditory canal, and maximal conductive hearing loss. Plain x-rays, magnetic resonance imaging, contrast-enhanced computed tomography, audiogram, and modified radical mastoidectomy. Radiologic and histopathologic diagnosis of a primary bone cyst of the temporal bone obstructing the external auditory canal with a retained cholesteatoma of the middle ear. Identification of an inflammatory bony cyst of the temporal bone with a retained cholesteatoma of the middle ear resulting in stenosis of the external auditory canal and maximal conductive hearing loss. Primary bone cyst of the temporal bone can lead to external auditory canal stenosis, middle ear cholesteatoma, and conductive hearing loss.

McRackan, Theodore R.; Carlson, Matthew L.; Reda, Fitsum A.; Noble, Jack H.; Rivas, Alejandro Author Information

Symptom: Conductive Hearing Loss after Cholesteatoma Surgery

Hearing is a special sense by which we perceive sound by detecting vibrations. Hearing ability is important for us to be able to interact, communicate and socialize. Sound is captured by the human ear which is a peripheral sensory organ composed of the external ear, middle ear and inner ear. The external ear serves to focus sound energy through the external auditory canal toward the tympanic membrane. The middle ear amplifies the sound energy via the ossicular chain which transfers sound vibrations to the inner ear. The inner ear includes the cochlea where mechano-electrical transduction of sound energy takes place. Humans perceive the vibrations of sound as transient auditory signals of vibratory mechanical forces transmitted through the external ear and middle ear, where they are transduced to neural signals in the inner ear passing via the auditory nerve to the central auditory system of the auditory cortex where the signal is processed and interpreted and passed on for perceptive processing. Understanding the central auditory processing in the central nervous system is essential to understanding how the sound information is discriminated and analysed to allow good communication. Any single problem in the peripheral or central auditory pathway may cause hearing impairment. Conductive hearing loss is defined as the hearing loss due to inappropriate mechanical transmission. Conditions that can cause conductive hearing loss are foreign body obstruction of the external auditory canal, perforation of the tympanic membrane, middle ear effusion, the destruction of ossicles, etc. Depending on the specific cause of conductive hearing loss, medical or surgical treatment can restore hearing. Sensorineural hearing loss refers the hearing loss caused by inner ear problems such as hair cell damage or neural degeneration. Unlike the conductive hearing loss, sensorineural hearing loss is usually permanent. Sensorineural hearing loss is divided into congenital and acquired. Congenital hearing loss may be caused by a congenital anomaly, a chromosomal syndrome or a congenital infection such as rubella or cytomegalovirus. Acquired sensorineural hearing loss might have numerous etiologic causes, including

Introduction: Since in 1995 the first case of absence of stapes was described, several cases were reported. However, its etiology remains unknown. Some authors suggest a genetic cause, without excludingthe possibility of embryopathy due to infections or chemical agents. The existence of an associated palatal cleft, in this case, reinforces the hypothesis of a multifactorial origin. Objective: Describe a rare condition in order to know how to suspect it. This condition is frequently associated with facial nerve malposition, that difficult the surgery and so, the most frequenttreatment is providing hearing aids or a bone anchored hearing aid. Case report: We present a case of a 10 years old girl who complains of left hearing loss since childhood, accompanied by tinnitus and frequent left ear otorrhea episodes. We studied symptoms, complementary exams, treatment and course. Discussion and conclusions: A middle ear malformation should be suspected with the presence of a history of conductive hearing loss since birth or more frequently between the 7 and 12years old, fixed-type, which often affects conversational or low frequencies, which are more intense than other acquired hearing loss, with no history of ear infections or without improvement despite different kinds of treatments, and witha family history of hearing loss. Conclusion: Congenital absence of stapes and oval window associated with anomalous course of the facial nerve is a rare entity. It presents as a conductive hearing loss non-progressive with 60 dB tone airthreshold, often presented during childhood. Diagnosis is based on clinical suspicion by a correct medical history and audiological examination, confirmed by Computed Tomography (CT) scan. The anomalous course of the facial nerve supports the diagnosis and guides treatment. Initial treatment with hearing aids provides good hearing gain with adequate adaptation.

1. Damage in the external, middle, or internal ear can contribute to the emergence of tinnitus because of the hearing loss it causes. 2. The two components of the external ear are the auricle and the outer auditory canal. (a) The occlusion of the ear canal produces an alteration in sound transmission that may cause tinnitus to develop. (b) Ear canal inflammation may cause tinnitus. 3. The middle ear is an impedance transformer and the site of several pathologies that all may cause tinnitus. (a) Acute otitis is accompanied by fever, strong pain in the ear, conductive hearing loss, and discharge from the ear. (b) Otitis media with effusion is a chronic presence of seromucous secretions in the middle ear cavity without signs of acute inflammation. (c) Otitis media is an inflammation of the middle ear causing conductive hearing loss. (d) Cholesteatoma is a mass of keratinizing squamous cells or epithelial debris that may occur in the middle ear cavity; it can erode body structures. (e) Otosclerosis involves a bony formation around the stapes, impeding its motion. 4. The first symptom of otosclerosis is often tinnitus. 5. Tinnitus often occurs in association with hearing loss of cochlear origin. (a) Acoustic trauma is one of the most common risk factors for the development of tinnitus and one of the major causes of permanent sensorineural hearing loss. (b) Administration of ototoxic drugs can cause hearing loss, tinnitus, and vertigo or dizziness. (c) Age-related changes can cause tinnitus and hearing loss. (d) Tinnitus is one of the three symptoms that define Meniere’s disease. (e) Changes (decrease) in cochlear blood perfusion can lead to cochlear damage with hearing loss and tinnitus. (f) Abrupt change in barometric pressure (barotraumas) can cause damage to the cochlea and may lead to tinnitus. 6. Hearing loss due to ear diseases may trigger a series of reactions in the central nervous system, which leads to the tinnitus. 7. Head trauma can lead to tinnitus, and balance disorders are very common after mild to severe head traumas.