Persistent Stapedial Artery, Oval Window Atresia and Congenital Stapes Agenesis—Case Report

IntroductionThe stapedial artery is an embryonic artery that transiently supplies the cranial vasculature of the human embryo. Postnatal persistence of the stapedial artery may cause conductive hearing loss and pulsatile...

The stapedial artery is an embryonic artery that transiently supplies the cranial vasculature of the human embryo. Postnatal persistence of the stapedial artery may cause conductive hearing loss and pulsatile tinnitus due to its course through the middle ear. We describe a patient with a persistent stapedial artery (PSA) managed by endovascular coil occlusion prior to stapedotomy. A 48-year-old woman presented with left-sided conductive hearing loss and pulsatile tinnitus. Ten years earlierthe patient had undergone explorative tympanoplasty, which was aborted due to a large PSA. Digital subtraction angiography was performed to confirm the anatomy and endovascular occlusion of the proximal PSA was achieved by deployment of coils. The pulsatile tinnitus improved immediately after the procedure. The size of the artery subsequently decreased and surgery could be performed with only a minor intraoperative bleeding. Successful stapedotomy resulted in postoperative normalization of her hearing with some minor residual tinnitus. Endovascular coil occlusion of a PSA is feasible and safe in patients with favorable anatomy and facilitates middle ear surgery. It decreases the size of the artery and minimizes the risk of intraoperative bleeding in patients with a large PSA. Thefuture role of this novel technique in the management of patients with PSA-related conductive hearing loss and pulsatile tinnitus remains to be determined.

Objective:To evaluate the outcome and per- and postoperative complications of the surgical management of patients with a persistent stapedial artery (PSA).Methods:A systemic literature search for reports on patients treated for pulsatile tinnitus and/or conductive hearing loss caused by a PSA was conducted of the PubMed and Embase databases using the terms “stapedial” and “artery.” Inclusion criteria were adequate description of the intervention and pre- and postoperative signs and symptoms. In addition, one case of a PSA, treated at VU University Medical Center Amsterdam, The Netherlands, was included in this series.Intervention:Middle ear surgery consisting of stapedotomy or stapedectomy, and/or transection of the PSA.Main Outcome Measures:Pre- and postoperative hearing levels, pre- and postoperative pulsatile tinnitus, and per- and postoperative complications.Results:Seventeen patients and 18 operated ears were evaluated (16 patients described in 14 articles and our case). Twelve out of 14 ears in which a stapedotomy or stapedectomy was initiated experienced improvement in hearing. In four cases pulsatile tinnitus was described pre- and postoperatively. In all four, pulsatile tinnitus subsided after transection of the PSA. Peroperative bleeding from the PSA was described in four patients, which could be controlled during the procedure. No significant postoperative sequelae were reported.Conclusions:In case of a PSA, improvement of conductive hearing loss is best achieved by stapes surgery, while pulsatile tinnitus is effectively treated with transection of the PSA. To date no long-term postoperative complications have been reported.

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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.

The number of potential options for rehabilitation of patients with conductive or mixed hearing loss is continually expanding. To be able to inform patients and other stakeholders, there is a need to identify and develop patient-centred outcomes for treatment of hearing loss. To identify outcome measures in the physical core area used when reporting the outcome after treatment of conductive and mixed hearing loss in adult patients. Systematic review. Systematic review of the literature related to reported physical outcome measures after treatment of mixed or conductive hearing loss without restrictions regarding type of intervention, treatment or device. Any measure reporting the physical outcome after treatment or intervention of mixed or conductive hearing loss was sought and categorised. The physical outcome measures that had been extracted were then grouped into domains. The literature search resulted in the identification of 1434 studies, of which 153 were selected for inclusion in the review. The majority (57%) of papers reported results from middle ear surgery, with the remainder reporting results from either bone conduction hearing devices or middle ear implants. Outcomes related to complications were categorised into 17 domains, whereas outcomes related to treatment success was categorised into 22 domains. The importance of these domains to patients and other stakeholders needs to be further explored in order to establish which of these domains are most relevant to interventions for conductive or mixed hearing loss. This will allow us to then assess which outcome measures are most suitable for inclusion in the core set.

The stapedial artery is an embryonic artery which disappears during the tenth week in utero, in human species. During its short life, this artery shapes the stapes and transforms the middle meningeal artery from the internal carotid artery to a branch of the external carotid system. Nevertheless, a persistent stapedial artery is seen in 0.2-4.8 per thousand of human adults. This persistence is usually asymptomatic but can sometimes cause pulsatile tinnitus or conductive hearing loss. Despite the risk of facial palsy, hearing loss and even hemiplegia argued by several authors, some surgeons have succeeded in coagulation without side effects. Reviewing the literature, we seek to enlighten the actual knowledge about the persistent stapedial artery to evaluate the risk to coagulate it. Embryologic studies explain the four types of persistent stapedial arteries: the hyoido-stapedial artery, the pharyngo-stapedial artery, the pharyngo-hyo-stapedial artery and aberrant internal carotid with persistent stapedial artery. Phylogenetic studies show that the stapedial artery persists in adulthood in many vertebrates. Its disappearance is therefore either a random effect or an adaptative convergence. This adaptation could be partially linked to the negative allometry of the stapes. Practically, the risk to coagulate a stapedial artery seems limited thanks to anastomoses, for example with the stylomastoid artery. The risk of hemiplegia reported is in fact an extrapolation of variation in rats' embryos. A persistent stapedial artery can therefore reasonably be coagulated, with special attention to the facial nerve, because the facial canal is always dehiscent where the artery penetrates.

Is Preoperative Computed Tomography Necessary or Useful for Primary Stapes Surgery?

ArticlesPlasticity of binaural interaction. II. Critical period and changes in midline responseB. M. Clopton, and M. S. SilvermanB. M. Clopton, and M. S. SilvermanPublished Online:01 Nov 1977https://doi.org/10.1152/jn.1977.40.6.1275MoreSectionsPDF (1 MB)Download PDF ToolsExport citationAdd to favoritesGet permissionsTrack citations ShareShare onFacebookTwitterLinkedInEmailWeChat Previous Back to Top Next Download PDF FiguresReferencesRelatedInformationCited ByA review of the effects of unilateral hearing loss on spatial hearingHearing ResearchImproved directional hearing of children with congenital unilateral conductive hearing loss implanted with an active bone-conduction implant or an active middle ear implantHearing ResearchEffects of Cochlear Implantation on Binaural Hearing in Adults With Unilateral Hearing Loss7 May 2018 | Trends in Hearing, Vol. 22Functional Impairments Due to Unilateral Deafness29 June 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 113, No. 12Monaural middle ear destruction in juvenile and adult mice: effects on responses to sound direction in the inferior colliculus ipsilateral to the intact earHearing Research, Vol. 174, No. 1-2Brainstem auditory evoked potentials in young children before and after tympanostomy tube placementInternational Journal of Pediatric Otorhinolaryngology, Vol. 25, No. 1-3Effects of recurrent otitis media in infancy on auditory perception and speechAmerican Journal of Otolaryngology, Vol. 13, No. 6Sound frequency and binaural response properties of single neurons in rat inferior colliculusHearing Research, Vol. 56, No. 1-2Changes in Spontaneous Activity and Cns Morphology Associated with Conductive and Sensorineural Hearing Loss in Chickens28 June 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 96, No. 3Effect of Conductive Hearing Loss and Middle Ear Surgery on Binaural Hearing29 June 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 95, No. 5Binaural interactions in cortical area AI of cats reared with unilateral atresia of the external ear canalHearing Research, Vol. 20, No. 3Some physiological consequences of neonatal cochlear destruction in the inferior colliculus of the gerbil, Meriones unguiculatusBrain Research, Vol. 306, No. 1-2Cholesteatoma Induction:Consequences of External Auditory Canal Ligation in Gerbils, Cats, Hamsters, Guinea Pigs, Mice and Rats8 July 2009 | Acta Oto-Laryngologica, Vol. 97, No. 3-4Auditory Brainstem Responses in Children with Early Recurrent Middle Ear Disease29 June 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 92, No. 3Age-dependent effects of acoustic deprivation on spherical cells of the rat anteroventral cochlear nucleusExperimental Neurology, Vol. 80, No. 1Development of the dorsal and ventral cochlear nuclei in rat and effects of acoustic deprivationDevelopmental Brain Research, Vol. 4, No. 1Scalp distribution of the auditory evoked brainstem potentials in the guinea pig during monaural and binaural stimulationHearing Research, Vol. 5, No. 2-3Short-term auditory deprivation: Effect on brainstem electrical responseHearing Research, Vol. 4, No. 3-4Plasticity of binaural interaction in the cat inferior colliculusBrain Research, Vol. 208, No. 1Plasticity of the Developing Auditory System29 June 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 89, No. 4Effects of Neonatal Conductive Hearing Loss on Brain Stem Auditory Nuclei28 June 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 88, No. 5Introduction4 December 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 88, No. 5_supplInfluence of Otitis Media on Hearing and Development4 December 2016 | Annals of Otology, Rhinology & Laryngology, Vol. 88, No. 5_supplEffects of monaural and binaural sound deprivation on cell development in the anteroventral cochlear nucleus of ratsExperimental Neurology, Vol. 64, No. 3 More from this issue > Volume 40Issue 6November 1977Pages 1275-1280 Copyright & PermissionsCopyright © 1977 the American Physiological Societyhttps://doi.org/10.1152/jn.1977.40.6.1275PubMed925729History Published online 1 November 1977 Published in print 1 November 1977 Metrics

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.

Plasticity of binaural interaction. I. Effect of early auditory deprivation

Oval window atresia (OWA) is a rare otologic condition often associated with a maximal conductive hearing loss, and variable ossicular and facial nerve canal (FNC) anomalies, which have contributed to suboptimal middle ear surgical outcomes. No grading scheme exists to detail the spectrum of associated temporal bone anomalies in OWA; therefore, our objectives were to complete an audiometric and radiographic review to characterize audiometric patterns of hearing loss, and refine the classification system for OWA to determine suitability for middle ear surgery. A retrospective audiometric and radiographic review was conducted at a pediatric tertiary care institution. Patients with OWA identified on temporal bone computerized tomography (CT) scans obtained from 01/2010 to 06/2024 were included. Audiological, radiological, and patient factors were analyzed. Thirty-one patients (48 ears) with OWA were identified. Across frequencies, the air-bone gap decreased significantly as frequency increased (ANOVA with pairwise comparisons, p < 0.001) due to a worsening of bone conduction thresholds and improvement in air conduction thresholds. The FNC was abnormal in 43/48 ears and was determined to overlay the oval window in 6 ears. Additional anomalies included inferiorly displaced, dehiscent, and duplicated canals. Ossicular anomalies were reported in 46/48 ears, and stapedial anomalies were most common. Our findings indicate OWA may manifest audiometrically with consistent and specific hearing loss characterized by a 60-80 dB ABG at lower frequencies that decreases above 2 kHz. CT findings of OWA show considerable variability. We propose a new classification system for OWA based on facial nerve position as this directly influences middle ear surgical feasibility.

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. 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. 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. 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.

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.

Background and ObjectivesConductive hearing loss is common among children and adults. This study aims at comparing the results of conductive hearing loss in summer and autumn.Subjects and MethodsPuretone audiometry and tympanometry tests were done for all patients who referred to the Iranian-based audiology center of Imam Khomeini Hospital in Ahvaz. Data on the patients with conductive or mixed hearing loss were analyzed. The impacts of season, age, and etiology of the disease were analyzed on the patients who visited the audiology clinic due to the conductive hearing loss in summer and autumn.ResultsOne hundred and fifty nine patients in summer and 123 patients in autumn had conductive or mixed hearing loss. Their age ranged from four to 82 years, with the average age of 35. The percentage of the patients, with acute otitis media and chronic otitis media (COM), who visited this clinic, was significantly higher than those with middle ear problems. COM and mastoid surgeries rate was higher in summer than autumn among adults.ConclusionsThere is no relationship between season and middle ear diseases between children and juveniles, but COM and mastoid problems are more common in summer among adults visiting this clinic. Most of the patients had mild conductive hearing loss and bilateral middle ear impairments.