Gradenigo’s syndrome is a complication of an otitis media infection and mastoiditis, involving three distinct manifestations: suppurative otitis media infection, facial pain compatible with the distribution of the trigeminal nerve and abducens nerve palsy.
Presentation
Gradenigo's syndrome, otherwise referred to as Gradenigo-Lannois syndrome and petrous apicitis, primarily arises as a complication following a case of otitis media infection and inflammation of the mastoid air cells of the temporal bone, that has extended to the petrous temporal bone [1] [2]. Its typical presentation involves pain that follows the distribution of the trigeminal nerve (5th cranial nerve), alongside palsy of the abducens nerve (6th cranial nerve) and otorrhea, the latter resulting from an otitis media infection [3]. Even though the aforementioned triad is considered to be the classical presentation of Gradenigo's syndrome, clinical characteristics of the infection are often diverse; photophobia, fever, excessive secretion of tears and a diminished corneal sensitivity may further complicate the clinical picture. Patients may also present with persistent headaches and some studies even report symptomatology consistent with facial and vestibulocochlear nerve pathology [4]. If left misdiagnosed or untreated, Gradenigo's syndrome can lead to serious neurological complications and death [5].
Given that Gradenigo's syndrome occurs as a complication of an original otitis media infection that has extended to involve the apex of the petrous temporal bone, the syndrome is currently considered a rare medical entity, since antibiotics started to be used in daily clinical practice.
Entire Body System
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Fever
She was without any preceding infectious symptoms including ear pain, sinus congestion, sore throat, and cough, and she denied fevers and chills. Examination demonstrated a right eye lateral gaze palsy and reproducible diplopia. [ncbi.nlm.nih.gov]
Other principal symptoms are photphobia, excessive lacrimation, fever, and reduced corneal sensitivity. The Gradenigo triad consists of otitis, abducens paralysis, and deep pain. [whonamedit.com]
[…] include: retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), abducens nerve palsy (sixth cranial nerve)[4] otitis media Other symptoms can include photophobia, excessive lacrimation, fever [en.wikipedia.org]
Even though the aforementioned triad is considered to be the classical presentation of Gradenigo's syndrome, clinical characteristics of the infection are often diverse; photophobia, fever, excessive secretion of tears and a diminished corneal sensitivity [symptoma.com]
Other symptoms can include photophobia, excessive lacrimation, fever, and reduced corneal sensitivity. The syndrome is usually caused by the spread of an infection into the petrous apex of the temporal bone. [ipfs.io]
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Crying
Upon admission the child was irritated, crying, complaining of otalgia to the right and pain in the right hemiface. His relatives denied the presence of vertiginous symptoms. [arquivosdeorl.org.br]
同 cat cry syndrome 猫鳴き症候群 ! クリミア・コンゴ出血熱ウイルス 櫛,稜 膨大部稜 鶏冠 ワニの涙症候群 クローン病 同 contralateral routing of signals(type) CROS(形)補聴器 ! hearing aid 同 shadow hearing 交叉聴取 ! 交叉性失語 同 cross-facial nerve graft 顔面神経交差移植術 ! [upslide.site]
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Malaise
He did not have nausea, vomiting, malaise or seizures. There was no ear pain or hearing loss. He had been taking oral cefuroxime prescribed by a general practitioner for the febrile illness during previous 5 days. [bmcpediatr.biomedcentral.com]
Fever and malaise precede neurological findings. The initial localising features are unilateral chemosis, periorbital oedema, and eyelid swelling. As retrobulbar pressure increases, ophthalmoplegia and exophthalmus ensue. [jnnp.bmj.com]
Respiratoric
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Aspiration
In more severe cases, a paracentesis (aspiration of fluids) or mastoidectomy may be needed. [en.wikipedia.org]
Despite the administration of high dose antibiotics the patient soon went on to develop stigmata of Vernet's syndrome (that is, involvement of IX, X, XI cranial nerves) followed by aspiration pneumonia. [doi.org]
Gastrointestinal
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Abdominal Mass
We report a case of a non-Hodgkin's lymphoma in a young woman presenting with an abdominal mass and an unusual instance of cranial nerve palsies mimicking Gradenigo's syndrome. [ncbi.nlm.nih.gov]
mass and an unusual instance of cranial nerve palsies mimicking Gradenigo’s syndrome. [doi.org]
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Recurrent Abdominal Pain
Browse recently published Learning/CME Learning/CME View all learning/CME CME Conservative Oxygen Therapy during Mechanical Ventilation in the ICU Case 8-2020: An 89-Year-Old Man with Recurrent Abdominal Pain and Bloody Stools Complications of Unsafe [nejm.org]
Eyes
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Lacrimation
Other principal symptoms are photphobia, excessive lacrimation, fever, and reduced corneal sensitivity. The Gradenigo triad consists of otitis, abducens paralysis, and deep pain. [whonamedit.com]
[…] syndrome include: retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), abducens nerve palsy (sixth cranial nerve)[4] otitis media Other symptoms can include photophobia, excessive lacrimation [en.wikipedia.org]
Other symptoms can include photophobia, excessive lacrimation, fever, and reduced corneal sensitivity. The syndrome is usually caused by the spread of an infection into the petrous apex of the temporal bone. [ipfs.io]
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Photophobia
[…] edit] Components of the syndrome include: retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), abducens nerve palsy (sixth cranial nerve)[4] otitis media Other symptoms can include photophobia [en.wikipedia.org]
Even though the aforementioned triad is considered to be the classical presentation of Gradenigo's syndrome, clinical characteristics of the infection are often diverse; photophobia, fever, excessive secretion of tears and a diminished corneal sensitivity [symptoma.com]
Other symptoms can include photophobia, excessive lacrimation, fever, and reduced corneal sensitivity. The syndrome is usually caused by the spread of an infection into the petrous apex of the temporal bone. [ipfs.io]
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Blepharitis
The ocular findings in individuals with Down syndrome include refractive errors, astigmatism, strabismus, nystagmus, amblyopia, cataracts, glaucoma, Brushfield spots, blepharitis, lacrimal obstructions, and corneal ectasias. [michiganorthopticprogram.com]
[…] arginine deiminase アルテルナリア症 alternariasis アルブミン albumin Alb アルベカシン arbekacin ABK (HBK) アレルギー allergy 即時型アレルギー immediate type allergy 遅延型アレルギー delayed type allergy 光アレルギー photo allergy ペニシリンアレルギー penicillin allergy 薬物アレルギー drug allergy アレルギー性眼瞼炎 allergic blepharitis [docsplayer.net]
Ears
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Otalgia
The middle ear and mastoid rhabdomyosarcoma may present a large variety of signs and symptoms, including facial paralysis, otorrhea, otalgia etc. (5). [arquivosdeorl.org.br]
A 45-years-old man initially complained of right otalgia and trigeminal neuralgia, while right abducens nerve paralysis appeared about two weeks after onset of acute otitis media. A CT scan showed an extradural abscess in the right petrous apex. [jstage.jst.go.jp]
Full Text A male aged 41 presented with a one-month duration of otalgia and deafness in the right ear, which was later associated with binocular diplopia, fever and slight non fetid whitish discharge from the ear. [elsevier.es]
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Tinnitus
[…] syndrome References: [8] Cerebellopontine angle syndrome Etiology : : most commonly vestibular schwannomas ( acoustic neuroma ), although other cerebellopontine angle tumors may result in the same presentation Clinical features Unilateral hearing loss Tinnitus [amboss.com]
[…] tympanic branch of the glossopharyngeal nerve (Jacobson's nerve) within the middle ear cavity Glomus jugulare - arise from paraganglia along the auricular branch of the vagus nerve (nerve of Arnold) in the jugular fossa Patients present with pulsatile tinnitus [spinwarp.ucsd.edu]
A History of Tinnitus. Tinnitus handbook. San Diego, CA: Singular Thomson Learning; 2000. p. 437-48 37. Stephens D, Orzan E. Galleti Di San Cataldo F: Giuseppe Gradenigo and his contributions to audiology. J Laryngol Otol. 1997. 111: 418-23 38. [surgicalneurologyint.com]
Progressive sensorineural hearing loss is the most common presenting symptom often associated with high pitched tinnitus. Tinnitus often subsides as hearing loss progresses to complete deafness. [jnnp.bmj.com]
病期分類 シュタール耳 歩行異常 標準失語症検査 標準マイクロホン 標準聴覚閾値 定在波 アブミ骨摘出術 アブミ骨神経 同 stapedius reflex アブミ骨筋反射 " ear)reflex stapedial tinnitus アブミ骨筋性耳鳴 stapedius(muscle)tendon アブミ骨筋腱 類 acoustic(middle ! [upslide.site]
Psychiatrical
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Dyslexia
[…] hearing loss drug inhalation dry cough dry nose dry powder inhaler dry swallow ductal papilloma ductus cochlearis(ラ) ductus reuniens(ラ) dumbbell-shaped tumor dura mater(ラ) dynein arm dysacusis dysarthria dysdiadochokinesis dysglossia dyskeratosis dyslalia dyslexia [upslide.site]
Face, Head & Neck
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Facial Pain
A 60-year-old woman presented to the emergency department with 7 days of right-sided headache, facial pain, and diplopia. She awoke with the headache and facial pain 7 days earlier. [ncbi.nlm.nih.gov]
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Sinus Pain
Painful ophthalmoplegia : partial/complete paresis of oculomotor nerve (third cranial nerve ), trochlear nerve (forth cranial nerve ), and abducens nerve (sixth cranial nerve ) Absent corneal reflex : paresis of the ophthalmic branch of the trigeminal [amboss.com]
Neurologic
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Headache
A 60-year-old woman presented to the emergency department with 7 days of right-sided headache, facial pain, and diplopia. She awoke with the headache and facial pain 7 days earlier. [ncbi.nlm.nih.gov]
A triad of Otitis media, left hemi cranial headache and left lateral rectus palsy consistent with Gradenigo's syndrome. [neuroradiologycases.com]
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Trigeminal Neuralgia
A 45-years-old man initially complained of right otalgia and trigeminal neuralgia, while right abducens nerve paralysis appeared about two weeks after onset of acute otitis media. A CT scan showed an extradural abscess in the right petrous apex. [jstage.jst.go.jp]
The clinical picture is of general interest, as the pain is in the form of a trigeminal neuralgia, except that it is limited to the first division of the fifth nerve and may persist for weeks or months after the discharge from the ear has ceased. [jamanetwork.com]
Trigeminal nerve (5th CN) involvement explains Trigeminal neuralgia - pain in the distribution of the trigeminal nerve manifest with hemicranial headache, hemi facial pain. 3. [neuroradiologycases.com]
neuralgia and ipsilateral abducens nerve palsy. [revistas.rcaap.pt]
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Excitement
Gradenigo’s Syndrome and Otitis Media We have discussed how exciting the management of otitis media can be already (see Acute Otitis Media and AOM and Cochlear Implants ). [carolinascoreconcepts.com]
[…] by · Published January 12, 2012 · Updated October 5, 2012 We have discussed how exciting the management of otitis media can be already (see Acute Otitis Media and AOM and Cochlear Implants). [pedemmorsels.com]
ネブライザー 頸部郭清術 頸部突出法 同 necrotizing labyrinthitis 壊死性内耳炎 " 壊死性血管炎 淋菌 ナイセリア属 nit 83 nemaline myopathy nematode neo-adjuvant chemotherapy neoglottal adjustment neoglottis neonatal screening nerve excitability test nerve growth factor nerve to stapedius nervus [upslide.site]
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Focal Neurologic Deficit
No other focal neurological deficit was present. Meningeal signs were negative. [hindawi.com]
Workup
The first step towards a precise diagnosis of Gradenigo's syndrome is a complete medical history, involving symptomatology and duration thereof and a confirmation of a recent or chronic otitis media infection. Manifestations that include an abducens nerve palsy, pain along the distribution of the trigeminal nerve and an otitis media infection that also involves the discharge of pus greatly direct suspicion towards Gradenigo's syndrome.
Imaging modalities that are used to diagnose the syndrome encompass a computerized tomography scan (CT scan) and a magnetic resonance imaging scan (MRI scan) of the temporal bone. The former usually illustrates hypodensity in the region of the petrous apex; the latter is expected to display hyperintensity on T2-weighted images and hypointensity on T1-weighted images. The infected region is enhanced in both the CT and the MRI scan. An MRI scan can also exhibit the pathology that underlies Gradenigo's syndrome and can range between cholesteatomas, malignant tumors, inflammatory granulomas and osteomyelitis [6] [7]. A single study also implemented diffusion-weighted MRI to aid in the depiction of abscesses and cholesteatomas in regions where diffusion is limited [6].
The differential diagnosis of Gradenigo's syndrome also entails various other pathologies that can assume the same symptomatology. Nasopharyngeal carcinoma and lymphoma of the petrous bone are two conditions whose symptomatology can greatly resemble that of Gradenigo's syndrome. Attention should be brought to the possibility of afebrile, non-otological manifestations after infectious petrositis, which could be misdiagnosed as a malignancy, with the consequence of antimicrobial treatment postponement influencing the prognosis [8].
Lumbar Puncture
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Turbid Cerebrospinal Fluid
Turbid cerebrospinal fluid could be drained. Follow-up could not be done as the patient refused treatment and was discharged against medical advice. [ncbi.nlm.nih.gov]
Treatment
Although there is little evidence to support the use of medical therapy in the treatment of Gradenigo's syndrome resulting from chronic ear disease, we here demonstrate successful conservative treatment of Gradenigo's syndrome following chronic otitis [ncbi.nlm.nih.gov]
Treatment[edit] The medical treatment is done with antibiotics: ceftriaxone plus metronidazole (which covers anaerobic bacteria). In more severe cases, a paracentesis (aspiration of fluids) or mastoidectomy may be needed. [en.wikipedia.org]
Treatment Awareness of the condition coupled with prompt investigations is required for early recognition. High dose antibiotic treatment, both systemic and topical, is the treatment of choice. [doi.org]
The authors describe in this article the case of a 7 year-old boy with this syndrome treated successfully with conservative treatment instead of surgical treatment. Keywords : Gradenigo's syndrome; petrositis; abducens palsy. [scielo.br]
Prognosis
Attention should be brought to the possibility of afebrile, non-otological manifestations after infectious petrositis, which could be misdiagnosed as a malignancy, with the consequence of antimicrobial treatment postponement influencing the prognosis. [symptoma.com]
A clinical study comprising 124 cases with special reference to clinical presentation and prognosis. Neurosurg Rev 1998;21:243-8. [Figure 1], [Figure 2] [cancerjournal.net]
Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the Intergroup Rhabdomyosarcoma Study. Cancer. 1987, 59:1846-1902. 12. [arquivosdeorl.org.br]
Prognosis Formerly, the prognosis was very poor with death a common occurrence. [patient.info]
Etiology
The majority of published cases involve children, and the most common etiology is otitis media. We report a case of a diabetic man who presented with repeating Gradenigo's syndrome symptoms due to NPC. [ncbi.nlm.nih.gov]
The most common etiology of this syndrome is an infection.[2] We reported a case that differs from these published reports: An NPC the etiology of Gradenigo's syndrome; the presence of recurrent symptoms of the syndrome persisted in a patient. [cancerjournal.net]
[…] to the trigeminal nerve References: [1] [2] Foster-Kennedy syndrome Orbital apex syndrome Cavernous sinus syndrome Etiology Cavernous sinus thrombosis (e.g., due to sinusitis ) Carotid-cavernous fistula Cavernous sinus tumors Carotid artery aneurysms [amboss.com]
Epidemiology
An awareness of its existence and appropriate levels of suspicion of the condition are necessary to prevent severe damage or death in those affected. [ 2, 3 ] Epidemiology There are no reliable recent figures. [patient.info]
Epidemiology Frequency Petrous apicitis was commonly encountered before the introduction of antibiotics. It now appears quite rarely. [emedicine.medscape.com]
壊死性胆嚢炎 necrotizing cholecystitis 壊死性腸炎 necrotizing enteritis(enterocolitis) 壊死性リンパ節炎 necrotizing lymphadenitis 壊疽 gangrene ガス壊疽 gas gangrene 糖尿病性壊疽 diabetic gangrene フルニエール壊疽 Fournier's gangrene 壊疽性口内炎 gangrenous stomatitis 衛星現象 satellite phenomenon 疫学 epidemiology [docsplayer.net]
Pathophysiology
The pathophysiology, therapy and differential diagnosis of this syndrome, which has become rare since the introduction of antibiotics, are discussed. [ncbi.nlm.nih.gov]
We hope to add new information to the existing literature and to aid in the understanding of the pathophysiology and management of this condition. [tandfonline.com]
Gradenigo classically described the following as the classic features of this syndrome: Discharging ear Retro orbital pain Adbucent nerve paralysis causing diplopia Causative factors: Uncontrolled mastoiditis Epidural abscess following mastoiditis Pathophysiology [drtbalu.co.in]
The potential role of hypocortisolism in the pathophysiology of stress-related bodily disorders. Psychoneuroendocrinology, 25(1), 1-35. Pagina 52 - In: Wall PD, Melzack R (eds), Textbook of pain. Churchill Livingstone, Edinburgh... [books.google.it]
Pathophysiology Bacteria travels from the middle ear to the mastoid air cells, which contain highly vascular marrow and are susceptible to infection [5]. [eyewiki.aao.org]
Prevention
There is little evidence to support increased use of antibiotics in acute otitis media to prevent this complication. [ncbi.nlm.nih.gov]
Suspicion of Gradenigo's syndrome (GS) is essential since early treatment is required to prevent permanent sequelae. [elsevier.es]
Timely management with intravenous antibiotics ( surgery) is needed to prevent intra-cranial complications. Publication Journal of the Pakistan Medical Association [ecommons.aku.edu]
Early recognition of condition is important to prevent intracranial complications like Meningitis, Intracranial abscess, Spread to skull base and involvement of IX, X, XI cranial nerves (Vernet’s syndrome), Prevertebral/parapharyngeal abscess, Spread [neuroradiologycases.com]
References
- Devic M, Boucher M, Raveau M. Some cases of Gradenigo-Lannois syndrome. Journal de médecine de Lyon. 1996;47 (96):537–547.
- Bléry M, Chagnon S, Picard A, Babin C. Cranial osteitis: a report on four cases, including a Gradenigo-Lannois syndrome (author's transl). Journal de radiologie. 1980;61(11):677–681.
- Motamed M, Kalan A. Gradenigo's syndrome. Postgraduate medical journal.2000;76(899):559-560.
- Sherman SC, Buchanan A. Gradenigo syndrome: a case report and review of a rare complication of otitis media. J Emerg Med. 2004;27:253–256.
- Valles JM, Fekete R. Gradenigo Syndrome: Unusual Consequence of Otitis Media. Case Rep Neurol. 2014;6(2):197–201.
- Ibrahim M, Shah G, Parmar H. Diffusion-weighted MRI identifies petrous apex abscess in Gradenigo syndrome. J Neuroophthalmol. 2010;30:34–36.
- Pedroso JL, de Aquino CC, Abrahão A, et al. Gradenigo's Syndrome: Beyond the Classical Triad of Diplopia, Facial Pain and Otorrhea. Case Rep Neurol. 2011;3:45–47.
- Yoong HS, Kiaang TK. Gradenigo's syndrome presenting as a tumor. Otolaryngol Head Neck Surg. 2006;135:821–822